BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell ...BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.展开更多
Genome sequencing has revealed frequent mutations in Ras homolog family member A(RHOA)among various cancers with unique aberrant profiles and pathogenic effects,especially in peripheral T-cell lymphoma(PTCL).The discr...Genome sequencing has revealed frequent mutations in Ras homolog family member A(RHOA)among various cancers with unique aberrant profiles and pathogenic effects,especially in peripheral T-cell lymphoma(PTCL).The discrete positional distribution and types of RHOA amino acid substitutions vary according to the tumor type,thereby leading to different functional and biological properties,which provide new insight into the molecular pathogenesis and potential targeted therapies for various tumors.However,the similarities and discrepancies in characteristics of RHOA mutations among various histologic subtypes of PTCL have not been fully elucidated.Herein we highlight the inconsistencies and complexities of the type and location of RHOA mutations and demonstrate the contribution of RHOA variants to the pathogenesis of PTCL by combining epigenetic abnormalities and activating multiple downstream pathways.The promising potential of targeting RHOA as a therapeutic modality is also outlined.This review provides new insight in the field of personalized medicine to improve the clinical outcomes for patients.展开更多
BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a common subtype of peripheral T-cell lymphoma.Approximately half of patients with AITL may concurrently present with hypergammaglobulinemia.Increased numbers of p...BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a common subtype of peripheral T-cell lymphoma.Approximately half of patients with AITL may concurrently present with hypergammaglobulinemia.Increased numbers of plasma cells in the bone marrow are commonly observed at diagnosis.These tumors mimic plasma cell myelomas,hindering a conundrum of clinical diagnoses and potentially delaying appropriate treatment.CASE SUMMARY A 78-year-old woman experienced poor appetite,weight loss of 5 kg,fatigue 2 months before presentation,and shortness of breath 2 d before presentation,but no fever or night sweats.Physical examination revealed splenomegaly and many palpable masses over the bilateral axillary regions,approximately>2 cm in size,with rubbery consistency and no tenderness.Blood tests revealed anemia and thrombocytopenia,lactate dehydrogenase level of 153 U/L,total protein level of 10.9 g/dL,albumin to globulin ratio of 0.2,and immunoglobulin G level more than the upper limit of 3000 mg/dL.The free kappa and lambda light chain concentrations were 451 and 614 mg/L,respectively.A pathological examination confirmed the diagnosis of AITL.The initial treatment was the cyclophosphamide,epirubicin,vincristine,and prednisolone regimen.Following this treatment,pleural effusion was controlled,and the patient was discharged in a stable condition and followed up in our outpatient department.CONCLUSION This report highlights the importance of differentiating reactive plasmacytosis from plasma cell myeloma in patients with hypergammaglobulinemia.A precise diagnosis of AITL requires a comprehensive evaluation,involving clinical,immunophenotypic,and histological findings conducted by a multidisciplinary team to ensure appropriate treatment.展开更多
BACKGROUND Gastrointestinal lymphoma typically arises in the stomach,small bowel,or colorectum and is usually a B-cell lymphoma.However,primary T-cell lymp-homas originating in the stomach are particularly rare.Gastri...BACKGROUND Gastrointestinal lymphoma typically arises in the stomach,small bowel,or colorectum and is usually a B-cell lymphoma.However,primary T-cell lymp-homas originating in the stomach are particularly rare.Gastric peripheral T-cell lymphoma-not otherwise specified(PTCL-NOS)is an extremely rare subtype.CASE SUMMARY We report a 63-year-old male presenting with epigastric pain.Esophagogastro-duodenoscopy revealed a large ulcerative lesion in the gastric cardia.Biopsy and immunohistochemical profiling confirmed PTCL-NOS.Imaging indicated stage II disease involving the stomach and intra-abdominal lymph nodes.The patient is planned to undergo cyclophosphamide,doxorubicin,vincristine,and prednisone or cyclophosphamide,doxorubicin,vincristine,prednisone,and etoposide chemo-therapy.CONCLUSION This case highlights the necessity of considering PTCL-NOS in differential diag-noses of gastric lesions.Comprehensive histopathological and immunohistoche-mical analysis is crucial for accurate diagnosis and guiding treatment.展开更多
BACKGROUND Monomorphic epithelial intestinal T-cell lymphoma(MEITL)is a rare type of peripheral T-cell lymphoma.The clinical manifestations are diarrhea,abdominal pain,perforation and an abdominal mass.CASE SUMMARY We...BACKGROUND Monomorphic epithelial intestinal T-cell lymphoma(MEITL)is a rare type of peripheral T-cell lymphoma.The clinical manifestations are diarrhea,abdominal pain,perforation and an abdominal mass.CASE SUMMARY We present a 52-year-old female patient who was diagnosed with MEITL.Further disease progression was observed after multiline chemotherapy.Eventually,the patient died of a severe infection.CONCLUSION MEITL is a rare intestinal primary T-cell lymphoma with aggressive behavior,a high risk of severe life-threatening complications,and a poor prognosis.展开更多
Due to an increasing incidence and more frequent recognition by endoscopists, gastrointestinal follicular lymphoma has been established as a variant of follicular lymphoma. However, due to its rarity, there are no est...Due to an increasing incidence and more frequent recognition by endoscopists, gastrointestinal follicular lymphoma has been established as a variant of follicular lymphoma. However, due to its rarity, there are no established guidelines on the optimal diagnostic strategy for patients with primary gastrointestinal follicular lymphoma or secondary gastrointestinal involvement of systemic follicular lymphoma. This review offers an overview and pitfalls to avoid during the initial diagnostic workup of this disease entity. Previously reported case reports, case series, and retrospective studies are reviewed and focus on the disease's endoscopic and histological features, the roles of computed tomography and positron emission tomography scanning, the clinical utility of the soluble interleukin-2 receptor, and the possible pathogenesis.展开更多
Double-balloon enteroscopy (DBE) and video capsule endoscopy are useful for the diagnosis of lymphoma in the small intestine. However, DBE cannot be safely performed in cases with passage disturbance due to wall thick...Double-balloon enteroscopy (DBE) and video capsule endoscopy are useful for the diagnosis of lymphoma in the small intestine. However, DBE cannot be safely performed in cases with passage disturbance due to wall thickening and stenosis. Additionally, video capsule endoscopy cannot be performed in such cases because of the risk of retention. Here, we report 4 cases of primary follicular lymphoma of the gastrointestinal tract that could be detected using 18F-fluoro-deoxyglucose positron emission tomography combined with computed tomography (PET-CT). The endoscopic findings of these 4 cases included lesions with wall thickening, which comprised macroscopically clusters of nodules, dense clusters of whitish granules or small nodules, fold thickening and ulcers with irregular margins that occupied the whole lumen with edematous mucosa. All patients fulfilled the World Health Organization grade 1 criteria. 18 F-fluorodeoxyglucose PET-CT can help predict the risks that may result from certain endoscopic examinations, such as DBE and video capsule endoscopy.展开更多
A 69-year-old Japanese female was diagnosed with primary intestinal follicular lymphoma. Esophagogas-troduodenoscopy with high-definition imaging revealed not only the typical feature of whitish polyps of up to 2 mm i...A 69-year-old Japanese female was diagnosed with primary intestinal follicular lymphoma. Esophagogas-troduodenoscopy with high-definition imaging revealed not only the typical feature of whitish polyps of up to 2 mm in diameter in the duodenal second and third portions, but also more detailed morphology, such as enlarged whitish villi and tiny whitish depositions. These findings appeared to reflect the pathological structures; infiltration of lymphoma cells into the villi were probably seen as enlargement of the villi, and the formation of lymphoid follicles were shown as opaque white spots or tiny white depositions. Thus, the above features might contribute to the distinct diagnosis of intestinal follicular lymphoma. This case indicates that routine esophagogastroduodenoscopy can visualize microsurface structures, which can be pathognomonic and help to diagnose intestinal follicular lymphoma, even without magnifying endoscopy.展开更多
Extranodal natural killer/T-cell lymphoma(ENKTL) is a distinct subtype of non-Hodgkin's lymphoma and is rare in the colon.Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature.I...Extranodal natural killer/T-cell lymphoma(ENKTL) is a distinct subtype of non-Hodgkin's lymphoma and is rare in the colon.Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature.In the present study,we report a 63-year-old male who suffered from intermittent bloody stools for 2 mo.He did not have fever,body weight loss or night sweat.Endoscopic and imaging studies revealed a 4.5-cm ulcerative mass in the ascending colon and a 3.0-cm polypoid,easy bleeding mass in the sigmoid colon,respectively.Thought to have double carcinoma of the colon,he received simultaneous right hemicolectomy and sigmoidectomy.The pathological diagnosis was a synchronous ENKTL(ascending colon) and adenocarcinoma(sigmoid colon).The literature on synchronous adenocarcinoma and malignant lymphoma of the colon was also reviewed.展开更多
Objective:To assess the efficacy and safety of the novel histone deacetylase inhibitor,chidamide,in combination with cyclophosphamide,doxorubicin,vincristine,etoposide,and prednisone(Chi-CHOEP)for untreated peripheral...Objective:To assess the efficacy and safety of the novel histone deacetylase inhibitor,chidamide,in combination with cyclophosphamide,doxorubicin,vincristine,etoposide,and prednisone(Chi-CHOEP)for untreated peripheral T-cell lymphoma(PTCL).Methods:A prospective,multicenter,single arm,phase 1 b/2 study was conducted.A total of 128 patients with untreated PTCL(18–70 years of age)were enrolled between March 2016 and November 2019,and treated with up to 6 cycles with the Chi-CHOEP regimen.In the phase 1 b study,3 dose levels of chidamide were evaluated and the primary endpoint was determination of the maximumtolerated dose and recommended phase 2 dose(RP2 D).The primary endpoint of the phase 2 study was 2-year progression-free survival(PFS).Results:Fifteen patients were enrolled in the phase 1 b study and the RP2 D for chidamide was determined to be 20 mg,twice a week.A total of 113 patients were treated at the RP2 D in the phase 2 study,and the overall response rate was 60.2%,with a complete response rate of 40.7%.At a median follow-up of 36 months,the median PFS was 10.7 months,with 1-,2-,and 3-year PFS rates of 49.9%,38.0%,and 32.8%,respectively.The Chi-CHOEP regimen was well-tolerated,with grade 3/4 neutropenia occurring in approximately two-thirds of the patients.No unexpected adverse events(AEs)were reported and the observed AEs were manageable.Conclusions:This large cohort phase 1 b/2 study showed that Chi-CHOEP was well-tolerated with modest efficacy in previously untreated PTCL patients.展开更多
In recent years,therapies for follicular lymphoma (FL) have steadily improved.A series of phase Ⅲ trials comparing the effect of rituximab with chemotherapy vs chemotherapy alone in treating FL have indicated signifi...In recent years,therapies for follicular lymphoma (FL) have steadily improved.A series of phase Ⅲ trials comparing the effect of rituximab with chemotherapy vs chemotherapy alone in treating FL have indicated significant improvements in progression-free survival (PFS) and overall survival.Recent studies have found that prolonged response durations and PFS were obtained with maintenance therapy using rituximab or interferon after completion of first line therapy.For patients with relapsed or refractory FL,phase Ⅱ studies have assessed the effectiveness of combination therapies using a Toll-like receptor-9 agonist (1018ISS),oblimersen sodium (a Bcl-2 antisense oligonucleotide),bendamustine,and rituximab,as well as veltuzumab,a new humanized anti-CD20 antibody,and epratuzumab.In addition,the effectiveness of yttrium-90 ibritumomab tiuxetan and iodine-131 tositumomab as radioimmunotherapies has been reported.Furthermore,three phase Ⅲ studies on an idiotype vaccine are near completion.Unfortunately,these vaccines,which appeared highly effective in phase Ⅰ and Ⅱ trials,do not appear to result in prolonged PFS.This report will summarize the current knowledge on therapies for treatment of FL,and will conclude with a brief discussion of feasiblefuture options for effective treatments.Lastly,we added descriptions of the management of gastrointestinal FL,which is considered to be controversial because it is rare.展开更多
ObjectiveTo observe the effect of taurine on immune function in mice with T-cell lymphoma during chemotherapy.MethodsA total of 40 C57BL/6 mice were selected and randomly divided into 4 groups, namely model group, che...ObjectiveTo observe the effect of taurine on immune function in mice with T-cell lymphoma during chemotherapy.MethodsA total of 40 C57BL/6 mice were selected and randomly divided into 4 groups, namely model group, chemotherapy group, taurine group and chemotherapy + taurine group, each containing 10 mice. Hypodermic injection was adopted to inoculate EL-4 cells in order to establish model of T-cell lymphoma. When the tumor achieved the size of 1 cm<sup>3</sup>, intervention treatments were given to the groups respectively. Mice in model group received 0.2 mL of normal saline which was intraperitoneally injected on Days 1, 8 and 15 with 3 weeks as a cycle; mice in chemotherapy group were administered with 80 mg/kg body weight of gemcitabine which was also intraperitoneally injected on Days 1, 8 and 15 with 3 weeks as a cycle; mice in taurine group were administered with 80 mg/kg body weight of taurine intraperitoneally injected daily for consecutive 8 d; mice in chemotherapy + taurine group were treated in the same manner as the mice in taurine group and chemotherapy group. Five mice were sacrificed at 2 and 3 weeks after intervention respectively, and the tumor tissues were collected and weighted after removal of auxiliary tissue, then the tumor inhibition rate was calculated. The thymus and spleen of mice sacrificed at 3 weeks after intervention were collected and weighted, and thymus and spleen indexes were calculated. Enzyme linked immunosorbent assay was used to detect the serum levels of IL-4, IL-10, IL-12 and IFN-γ in mice of each group.ResultsThe tumor weights in chemotherapy group, taurine group and chemotherapy + taurine group after 2 and 3 weeks of treatment were significantly lower than that in model group (P < 0.05); the tumor weight in chemotherapy + taurine group after 2 and 3 weeks of treatment was significantly lower than that in chemotherapy group (P < 0.05); the tumor inhibition rate in chemotherapy + taurine group was significantly higher than that in chemotherapy group and taurine group (P < 0.05); the thymus and spleen indexes in taurine group and chemotherapy + taurine group were significantly higher than those in chemotherapy group and model group (P < 0.05); the thymus and spleen indexes in chemotherapy group were significantly lower than those in model group (P < 0.05); after 3 weeks of treatment, the serum levels of IL-4, IL-12 and IFN-γ in chemotherapy group, taurine group and chemotherapy + taurine group were significantly lower than those in model group (P < 0.05); the IL-4 level in taurine group and chemotherapy + taurine group was significantly lower than that in chemotherapy group (P < 0.05); the serum level of IL-10 in chemotherapy group and chemotherapy + taurine group was significantly higher than that in model group and taurine group (P < 0.05); the serum level of IFN-γ in taurine group and chemotherapy + taurine group was significantly lower than that in model group and chemotherapy group (P < 0.05); after treatment of 3 weeks, the serum levels of IL-4 and IL-10 in chemotherapy group, taurine group and chemotherapy + taurine group were significantly lower than those in model group (P < 0.05), and IL-12 level was significantly higher than that in model group (P < 0.05); the level of IFN-γ in taurine group and chemotherapy + taurine group was significantly higher than that in model group (P < 0.05), while the level of IFN-γ in chemotherapy group was significantly lower than that in the other 3 groups (P < 0.05).ConclusionsTaurine can effectively enhance the immune function of mice with T-cell lymphoma during chemotherapy, reduce the toxicity of chemotherapy.展开更多
BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is a rare primary intestinal T-cell lymphoma,previously known as enteropathy-associated T-cell lymphoma type II.MEITL is an aggressive T-cell lym...BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is a rare primary intestinal T-cell lymphoma,previously known as enteropathy-associated T-cell lymphoma type II.MEITL is an aggressive T-cell lymphoma with a poor prognosis and high mortality rate.The known major complications of MEITL are intestinal perforation and obstruction.Here,we present a case of MEITL that was diagnosed following upper gastrointestinal bleeding from an ulcerative duodenal lesion,with recurrence-free survival for 5 years.CASE SUMMARY A 68-year-old female was admitted to our hospital with melena and mild anemia.An urgent esophagogastroduodenoscopy(EGD)revealed bleeding from an ulcerative lesion in the transverse part of the duodenum,for which hemostatic treatment was performed.MEITL was diagnosed following repeated biopsies of the lesion,and cyclophosphamide,doxorubicin,vincristine,and prednisone(CHOP)chemotherapy was administered.She achieved complete remission after eight full cycles of CHOP therapy.At the last follow-up examination,EGD revealed a scarred ulcer and 18Fluorodeoxyglucose(18FDG)positron emission tomography/computed tomography showed no abnormal FDG accumulation.The patient has been in complete remission for 68 mo after initial diagnosis.CONCLUSION To rule out MEITL,it is important to carefully perform histological examination when bleeding from a duodenal ulcer is observed.展开更多
BACKGROUND Duodenal-type follicular lymphoma(D-FL)has been recognized as a rare entity that accounts for approximately 4%of primary gastrointestinal lymphomas.D-FL follows an indolent clinical course compared with com...BACKGROUND Duodenal-type follicular lymphoma(D-FL)has been recognized as a rare entity that accounts for approximately 4%of primary gastrointestinal lymphomas.D-FL follows an indolent clinical course compared with common nodal FL and is generally considered to have a better prognosis.Therefore,the“watch and wait”approach is frequently adopted as the treatment method.Alternatively,there is an option to actively intervene in D-FL.However,the long-term outcomes of such cases are poorly understood.AIM To clarify the clinical outcomes after long-term follow-up in cases of D-FL with treatment intervention.METHODS We retrospectively analyzed patients who met the following criteria:the lesion was confirmed by endoscopy,the diagnosis of D-FL was confirmed histopathologically,and the patient was followed-up for more than 10 years after the intervention at our center.RESULTS We identified 5 cases of D-FL.Two patients showed a small amount of bone marrow involvement(Stage IV).Rituximab was used as a treatment for remission in all 5 patients.It was also used in combination with chemotherapy in 2 Stage IV patients as well as for maintenance treatment.Radiation therapy was performed in 2 cases,which was followed by complete remission(CR).Eventually,all 5 patients achieved CR and survived for more than 10 years.However,3 patients experienced recurrence.One patient achieved a second CR by retreatment,and in another case,the lesion showed spontaneous disappearance.The remaining patient had systemic widespread recurrence 13 years after the first CR.Biopsy results suggested that the FL lesions were transformed into diffuse large B-cell lymphoma.The patient died 4 years later despite receiving various chemotherapies.CONCLUSION In this study,the treatment for patients of D-FL in Stage IV was successful.In the future,criteria for how to treat“advanced”D-FL should be established based on additional cases.This study of patients with D-FL indicates that whole-body follow-up examinations should continue for a long time due to a fatal recurrence 13 years after reaching CR.展开更多
This study investigated the effects of miRNA-155 on malignant biological characteristics of NK/T-cell lymphoma cell lines and the possible mechanism. The expression of miRNA-155 was detected in lymphoma cell lines fro...This study investigated the effects of miRNA-155 on malignant biological characteristics of NK/T-cell lymphoma cell lines and the possible mechanism. The expression of miRNA-155 was detected in lymphoma cell lines from different sources (SNK-6, YTS, Jurkat and DOHH2) by real-time PCR. Lentiviral vectors (pLL3.7) that could overexpress or downexpress miRNA-155 were constructed. Recombinant lentiviral particles were prepared and purified, and their titers determined. The expression of miRNA-155 in the infected SNK-6 cells and the cell proliferation were detected by PCR and CCK-8, respectively. Flow cytometry was used to determine the apoptosis of infected SNK-6 cells. The target of miRNA155 was predicted from Targetscan website. The effect of miRNA155 on FOXO3a expression was examined by Western blotting. The results showed that among the human NK/T-cell lymphoma cell lines SNK-6, YTS, Jurkat and DOHH2, the expression of miRNA-155 was highest in SNK-6. The infection efficiency of the recombinant lentivirns in SNK-6 was more than 70% at multiplicity of infection (MOI) of 100. The expression of miRNA-155 was significantly increased in SNK-6 cells infected by lentivirus vectors with high expression of miRNA-155 (4 times higher than the control group), and profoundly decreased in those infected with lentivirnses with low expression of miRNA-155. The proliferation of letivirns-infected SNK-6 cells was decreased as the expression of miRNA-155 reduced. The apoptosis rate was increased with the reduction in the expression of miRNA-155. FOXO3a was found to be a possible target of miRNA155, as suggested by Targetscan website. Western blotting showed that the expression of FOXO3a was significantly elevated in SNK-6 cells with miRNA-155 inhibition. It was concluded that reduction in miRNA-155 expression can inhibit the proliferation of SNK-6 lymphoma cells and promote their apoptosis, which may be associated with regulation of FOXO3a gene.展开更多
AIM:To study the diagnostic value of immunoglobulin heavy chain(IgH)and T-cell receptorγ (TCR-γ)gene monoclonal rearrangements in primary gastric lymphoma(PGL).METHODS:A total of 48 patients with suspected PGL at ou...AIM:To study the diagnostic value of immunoglobulin heavy chain(IgH)and T-cell receptorγ (TCR-γ)gene monoclonal rearrangements in primary gastric lymphoma(PGL).METHODS:A total of 48 patients with suspected PGL at our hospital were prospectively enrolled in this study from January 2009 to December 2011.The patients were divided into three groups(a PGL group,a gastric linitis plastica group,and a benign gastric ulcer group)based on the pathological results(gastric mucosal specimens obtained by endoscopy or surgery)and follow-up.Endoscopic ultrasonography(EUS)and EUSguided biopsy were performed in all the patients.The tissue specimens were used for histopathological examination and for IgH and TCR-γ gene rearrangement polymerase chain reaction analyses.RESULTS:EUS and EUS-guided biopsy were successfully performed in all 48 patients.In the PGL group(n=21),monoclonal IgH gene rearrangements were detected in 14(66.7%)patients.A positive result for each set of primers was found in 12(57.1%),8(38.1%),and 4(19.0%)cases using FR1/JH,FR2/JH,and FR3/JH primers,respectively.Overall,12(75%)patients with mucosal-associated lymphoid tissue lymphoma(n=16)and 2(40%)patients with diffuse large B-cell lymphoma(n=5)were positive for monoclonal IgH gene rearrangements.No patients in the gastric linitis plastica group(n=17)and only one(10%)patient in the benign gastric ulcer group(n=10)were positive for a monoclonal IgH gene rearrangement.No TCRgene monoclonal rearrangements were detected.The sensitivity of monoclonal IgH gene rearrangements was 66.7%for a PGL diagnosis,and the specificity was96.4%.In the PGL group,8(100%)patients with stage IIE PGL(n=8)and 6(46.1%)patients with stage IE PGL(n=13)were positive for monoclonal IgH gene rearrangements.CONCLUSION:IgH gene rearrangements may be associated with PGL staging and may be useful for the diagnosis of PGL and for differentiating between PGL and gastric linitis plastica.展开更多
T-cell/histiocyte-rich large B-cell lymphoma is uncommon in children population. There were few cases reported in the literature with wide range clinical presentations including advanced stage, and more involvement of...T-cell/histiocyte-rich large B-cell lymphoma is uncommon in children population. There were few cases reported in the literature with wide range clinical presentations including advanced stage, and more involvement of liver, spleen and bone marrow. Head and neck lymphadenopathy tends to present in younger children. We report a case of 10-year-old boy who initially presented intermittent fever, headaches and neck lymphadenopathy. Subsequently, he developed diffuse lymphadenopathy and hepatosplenomegaly. T-cell/histiocyte-rich large B-cell lymphoma was diagnosed on a cervical lymph node biopsy. Cervical lymphadenopathy in this age group is most commonly reactive or nonmalignant processes. Lymphoma is much less frequent; mainly are non-Hodgkin lymphomas. However, a subset of large B-cell lymphoma called T-cell/histiocyte-rich B-cell lymphoma is rare in children.展开更多
Adoptive cellular immunotherapy with chimeric antigen receptor(CAR)T cells has emerged as a novel modality for treating relapsed and/or refractory B-cell non-Hodgkin lymphoma(B-NHL).With increasing approval of CAR T-c...Adoptive cellular immunotherapy with chimeric antigen receptor(CAR)T cells has emerged as a novel modality for treating relapsed and/or refractory B-cell non-Hodgkin lymphoma(B-NHL).With increasing approval of CAR T-cell products and advances in CAR T cell therapy,CAR T cells are expected to be used in a growing number of cases.However,CAR T-cell-associated toxicities can be severe or even fatal,thus compromising the survival benefit from this therapy.Standardizing and studying the clinical management of these toxicities are imperative.In contrast to other hematological malignancies,such as acute lymphoblastic leukemia and multiple myeloma,anti-CD19 CAR T-cell-associated toxicities in B-NHL have several distinctive features,most notably local cytokine-release syndrome(CRS).However,previously published guidelines have provided few specific recommendations for the grading and management of toxicities associated with CAR T-cell treatment for B-NHL.Consequently,we developed this consensus for the prevention,recognition,and management of these toxicities,on the basis of published literature regarding the management of anti-CD19 CAR T-cell-associated toxicities and the clinical experience of multiple Chinese institutions.This consensus refines a grading system and classification of CRS in B-NHL and corresponding measures for CRS management,and delineates comprehensive principles and exploratory recommendations for managing anti-CD19 CAR T-cell-associated toxicities in addition to CRS.展开更多
Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage ...Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital.The median age of these patients was 53.36±14.45 years.Female-male ratio was 1:1.2.The average course of disease was 10.82±3.37 years.All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51(40-74) Gy in an average of 24.55±5.57(20-40) fractions,5 fractions per week.Results The median follow-up time was 55.27±29.3(13-103) months.No severe acute or chronic side effects of irradiation were observed.Complete clinical response(CR) rate of the radiated sites was 54.5%(6/11),partial response(PR) rate was 36.4%(4/11),and the overall response rate(CR+PR) was 90.9%.One patient showed no response.Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.展开更多
文摘BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.
基金This work was supported by the Natural Science Foundation of Guangdong Province(Grant No.2019A1515011354).
文摘Genome sequencing has revealed frequent mutations in Ras homolog family member A(RHOA)among various cancers with unique aberrant profiles and pathogenic effects,especially in peripheral T-cell lymphoma(PTCL).The discrete positional distribution and types of RHOA amino acid substitutions vary according to the tumor type,thereby leading to different functional and biological properties,which provide new insight into the molecular pathogenesis and potential targeted therapies for various tumors.However,the similarities and discrepancies in characteristics of RHOA mutations among various histologic subtypes of PTCL have not been fully elucidated.Herein we highlight the inconsistencies and complexities of the type and location of RHOA mutations and demonstrate the contribution of RHOA variants to the pathogenesis of PTCL by combining epigenetic abnormalities and activating multiple downstream pathways.The promising potential of targeting RHOA as a therapeutic modality is also outlined.This review provides new insight in the field of personalized medicine to improve the clinical outcomes for patients.
文摘BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a common subtype of peripheral T-cell lymphoma.Approximately half of patients with AITL may concurrently present with hypergammaglobulinemia.Increased numbers of plasma cells in the bone marrow are commonly observed at diagnosis.These tumors mimic plasma cell myelomas,hindering a conundrum of clinical diagnoses and potentially delaying appropriate treatment.CASE SUMMARY A 78-year-old woman experienced poor appetite,weight loss of 5 kg,fatigue 2 months before presentation,and shortness of breath 2 d before presentation,but no fever or night sweats.Physical examination revealed splenomegaly and many palpable masses over the bilateral axillary regions,approximately>2 cm in size,with rubbery consistency and no tenderness.Blood tests revealed anemia and thrombocytopenia,lactate dehydrogenase level of 153 U/L,total protein level of 10.9 g/dL,albumin to globulin ratio of 0.2,and immunoglobulin G level more than the upper limit of 3000 mg/dL.The free kappa and lambda light chain concentrations were 451 and 614 mg/L,respectively.A pathological examination confirmed the diagnosis of AITL.The initial treatment was the cyclophosphamide,epirubicin,vincristine,and prednisolone regimen.Following this treatment,pleural effusion was controlled,and the patient was discharged in a stable condition and followed up in our outpatient department.CONCLUSION This report highlights the importance of differentiating reactive plasmacytosis from plasma cell myeloma in patients with hypergammaglobulinemia.A precise diagnosis of AITL requires a comprehensive evaluation,involving clinical,immunophenotypic,and histological findings conducted by a multidisciplinary team to ensure appropriate treatment.
基金Supported by 2024 Kangwon National University Hospital Grant,No.KNUH_2024_02_06.
文摘BACKGROUND Gastrointestinal lymphoma typically arises in the stomach,small bowel,or colorectum and is usually a B-cell lymphoma.However,primary T-cell lymp-homas originating in the stomach are particularly rare.Gastric peripheral T-cell lymphoma-not otherwise specified(PTCL-NOS)is an extremely rare subtype.CASE SUMMARY We report a 63-year-old male presenting with epigastric pain.Esophagogastro-duodenoscopy revealed a large ulcerative lesion in the gastric cardia.Biopsy and immunohistochemical profiling confirmed PTCL-NOS.Imaging indicated stage II disease involving the stomach and intra-abdominal lymph nodes.The patient is planned to undergo cyclophosphamide,doxorubicin,vincristine,and prednisone or cyclophosphamide,doxorubicin,vincristine,prednisone,and etoposide chemo-therapy.CONCLUSION This case highlights the necessity of considering PTCL-NOS in differential diag-noses of gastric lesions.Comprehensive histopathological and immunohistoche-mical analysis is crucial for accurate diagnosis and guiding treatment.
基金Supported by Zhejiang Province Traditional Chinese Medicine Science and Technology Project,No.2024ZL1296.
文摘BACKGROUND Monomorphic epithelial intestinal T-cell lymphoma(MEITL)is a rare type of peripheral T-cell lymphoma.The clinical manifestations are diarrhea,abdominal pain,perforation and an abdominal mass.CASE SUMMARY We present a 52-year-old female patient who was diagnosed with MEITL.Further disease progression was observed after multiline chemotherapy.Eventually,the patient died of a severe infection.CONCLUSION MEITL is a rare intestinal primary T-cell lymphoma with aggressive behavior,a high risk of severe life-threatening complications,and a poor prognosis.
文摘Due to an increasing incidence and more frequent recognition by endoscopists, gastrointestinal follicular lymphoma has been established as a variant of follicular lymphoma. However, due to its rarity, there are no established guidelines on the optimal diagnostic strategy for patients with primary gastrointestinal follicular lymphoma or secondary gastrointestinal involvement of systemic follicular lymphoma. This review offers an overview and pitfalls to avoid during the initial diagnostic workup of this disease entity. Previously reported case reports, case series, and retrospective studies are reviewed and focus on the disease's endoscopic and histological features, the roles of computed tomography and positron emission tomography scanning, the clinical utility of the soluble interleukin-2 receptor, and the possible pathogenesis.
文摘Double-balloon enteroscopy (DBE) and video capsule endoscopy are useful for the diagnosis of lymphoma in the small intestine. However, DBE cannot be safely performed in cases with passage disturbance due to wall thickening and stenosis. Additionally, video capsule endoscopy cannot be performed in such cases because of the risk of retention. Here, we report 4 cases of primary follicular lymphoma of the gastrointestinal tract that could be detected using 18F-fluoro-deoxyglucose positron emission tomography combined with computed tomography (PET-CT). The endoscopic findings of these 4 cases included lesions with wall thickening, which comprised macroscopically clusters of nodules, dense clusters of whitish granules or small nodules, fold thickening and ulcers with irregular margins that occupied the whole lumen with edematous mucosa. All patients fulfilled the World Health Organization grade 1 criteria. 18 F-fluorodeoxyglucose PET-CT can help predict the risks that may result from certain endoscopic examinations, such as DBE and video capsule endoscopy.
文摘A 69-year-old Japanese female was diagnosed with primary intestinal follicular lymphoma. Esophagogas-troduodenoscopy with high-definition imaging revealed not only the typical feature of whitish polyps of up to 2 mm in diameter in the duodenal second and third portions, but also more detailed morphology, such as enlarged whitish villi and tiny whitish depositions. These findings appeared to reflect the pathological structures; infiltration of lymphoma cells into the villi were probably seen as enlargement of the villi, and the formation of lymphoid follicles were shown as opaque white spots or tiny white depositions. Thus, the above features might contribute to the distinct diagnosis of intestinal follicular lymphoma. This case indicates that routine esophagogastroduodenoscopy can visualize microsurface structures, which can be pathognomonic and help to diagnose intestinal follicular lymphoma, even without magnifying endoscopy.
基金Supported by The Buddhist Dalin Tzu-Chi General Hospital
文摘Extranodal natural killer/T-cell lymphoma(ENKTL) is a distinct subtype of non-Hodgkin's lymphoma and is rare in the colon.Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature.In the present study,we report a 63-year-old male who suffered from intermittent bloody stools for 2 mo.He did not have fever,body weight loss or night sweat.Endoscopic and imaging studies revealed a 4.5-cm ulcerative mass in the ascending colon and a 3.0-cm polypoid,easy bleeding mass in the sigmoid colon,respectively.Thought to have double carcinoma of the colon,he received simultaneous right hemicolectomy and sigmoidectomy.The pathological diagnosis was a synchronous ENKTL(ascending colon) and adenocarcinoma(sigmoid colon).The literature on synchronous adenocarcinoma and malignant lymphoma of the colon was also reviewed.
基金funded by the National Natural Science Foundation of China(Grant No.81970188)the Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(Grant No.2016-12M-1-001)。
文摘Objective:To assess the efficacy and safety of the novel histone deacetylase inhibitor,chidamide,in combination with cyclophosphamide,doxorubicin,vincristine,etoposide,and prednisone(Chi-CHOEP)for untreated peripheral T-cell lymphoma(PTCL).Methods:A prospective,multicenter,single arm,phase 1 b/2 study was conducted.A total of 128 patients with untreated PTCL(18–70 years of age)were enrolled between March 2016 and November 2019,and treated with up to 6 cycles with the Chi-CHOEP regimen.In the phase 1 b study,3 dose levels of chidamide were evaluated and the primary endpoint was determination of the maximumtolerated dose and recommended phase 2 dose(RP2 D).The primary endpoint of the phase 2 study was 2-year progression-free survival(PFS).Results:Fifteen patients were enrolled in the phase 1 b study and the RP2 D for chidamide was determined to be 20 mg,twice a week.A total of 113 patients were treated at the RP2 D in the phase 2 study,and the overall response rate was 60.2%,with a complete response rate of 40.7%.At a median follow-up of 36 months,the median PFS was 10.7 months,with 1-,2-,and 3-year PFS rates of 49.9%,38.0%,and 32.8%,respectively.The Chi-CHOEP regimen was well-tolerated,with grade 3/4 neutropenia occurring in approximately two-thirds of the patients.No unexpected adverse events(AEs)were reported and the observed AEs were manageable.Conclusions:This large cohort phase 1 b/2 study showed that Chi-CHOEP was well-tolerated with modest efficacy in previously untreated PTCL patients.
文摘In recent years,therapies for follicular lymphoma (FL) have steadily improved.A series of phase Ⅲ trials comparing the effect of rituximab with chemotherapy vs chemotherapy alone in treating FL have indicated significant improvements in progression-free survival (PFS) and overall survival.Recent studies have found that prolonged response durations and PFS were obtained with maintenance therapy using rituximab or interferon after completion of first line therapy.For patients with relapsed or refractory FL,phase Ⅱ studies have assessed the effectiveness of combination therapies using a Toll-like receptor-9 agonist (1018ISS),oblimersen sodium (a Bcl-2 antisense oligonucleotide),bendamustine,and rituximab,as well as veltuzumab,a new humanized anti-CD20 antibody,and epratuzumab.In addition,the effectiveness of yttrium-90 ibritumomab tiuxetan and iodine-131 tositumomab as radioimmunotherapies has been reported.Furthermore,three phase Ⅲ studies on an idiotype vaccine are near completion.Unfortunately,these vaccines,which appeared highly effective in phase Ⅰ and Ⅱ trials,do not appear to result in prolonged PFS.This report will summarize the current knowledge on therapies for treatment of FL,and will conclude with a brief discussion of feasiblefuture options for effective treatments.Lastly,we added descriptions of the management of gastrointestinal FL,which is considered to be controversial because it is rare.
基金supported by Youth Scientific Research Project of Fujian Provincial Health and Family Planning Commission(No.2017-1-57)
文摘ObjectiveTo observe the effect of taurine on immune function in mice with T-cell lymphoma during chemotherapy.MethodsA total of 40 C57BL/6 mice were selected and randomly divided into 4 groups, namely model group, chemotherapy group, taurine group and chemotherapy + taurine group, each containing 10 mice. Hypodermic injection was adopted to inoculate EL-4 cells in order to establish model of T-cell lymphoma. When the tumor achieved the size of 1 cm<sup>3</sup>, intervention treatments were given to the groups respectively. Mice in model group received 0.2 mL of normal saline which was intraperitoneally injected on Days 1, 8 and 15 with 3 weeks as a cycle; mice in chemotherapy group were administered with 80 mg/kg body weight of gemcitabine which was also intraperitoneally injected on Days 1, 8 and 15 with 3 weeks as a cycle; mice in taurine group were administered with 80 mg/kg body weight of taurine intraperitoneally injected daily for consecutive 8 d; mice in chemotherapy + taurine group were treated in the same manner as the mice in taurine group and chemotherapy group. Five mice were sacrificed at 2 and 3 weeks after intervention respectively, and the tumor tissues were collected and weighted after removal of auxiliary tissue, then the tumor inhibition rate was calculated. The thymus and spleen of mice sacrificed at 3 weeks after intervention were collected and weighted, and thymus and spleen indexes were calculated. Enzyme linked immunosorbent assay was used to detect the serum levels of IL-4, IL-10, IL-12 and IFN-γ in mice of each group.ResultsThe tumor weights in chemotherapy group, taurine group and chemotherapy + taurine group after 2 and 3 weeks of treatment were significantly lower than that in model group (P < 0.05); the tumor weight in chemotherapy + taurine group after 2 and 3 weeks of treatment was significantly lower than that in chemotherapy group (P < 0.05); the tumor inhibition rate in chemotherapy + taurine group was significantly higher than that in chemotherapy group and taurine group (P < 0.05); the thymus and spleen indexes in taurine group and chemotherapy + taurine group were significantly higher than those in chemotherapy group and model group (P < 0.05); the thymus and spleen indexes in chemotherapy group were significantly lower than those in model group (P < 0.05); after 3 weeks of treatment, the serum levels of IL-4, IL-12 and IFN-γ in chemotherapy group, taurine group and chemotherapy + taurine group were significantly lower than those in model group (P < 0.05); the IL-4 level in taurine group and chemotherapy + taurine group was significantly lower than that in chemotherapy group (P < 0.05); the serum level of IL-10 in chemotherapy group and chemotherapy + taurine group was significantly higher than that in model group and taurine group (P < 0.05); the serum level of IFN-γ in taurine group and chemotherapy + taurine group was significantly lower than that in model group and chemotherapy group (P < 0.05); after treatment of 3 weeks, the serum levels of IL-4 and IL-10 in chemotherapy group, taurine group and chemotherapy + taurine group were significantly lower than those in model group (P < 0.05), and IL-12 level was significantly higher than that in model group (P < 0.05); the level of IFN-γ in taurine group and chemotherapy + taurine group was significantly higher than that in model group (P < 0.05), while the level of IFN-γ in chemotherapy group was significantly lower than that in the other 3 groups (P < 0.05).ConclusionsTaurine can effectively enhance the immune function of mice with T-cell lymphoma during chemotherapy, reduce the toxicity of chemotherapy.
文摘BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is a rare primary intestinal T-cell lymphoma,previously known as enteropathy-associated T-cell lymphoma type II.MEITL is an aggressive T-cell lymphoma with a poor prognosis and high mortality rate.The known major complications of MEITL are intestinal perforation and obstruction.Here,we present a case of MEITL that was diagnosed following upper gastrointestinal bleeding from an ulcerative duodenal lesion,with recurrence-free survival for 5 years.CASE SUMMARY A 68-year-old female was admitted to our hospital with melena and mild anemia.An urgent esophagogastroduodenoscopy(EGD)revealed bleeding from an ulcerative lesion in the transverse part of the duodenum,for which hemostatic treatment was performed.MEITL was diagnosed following repeated biopsies of the lesion,and cyclophosphamide,doxorubicin,vincristine,and prednisone(CHOP)chemotherapy was administered.She achieved complete remission after eight full cycles of CHOP therapy.At the last follow-up examination,EGD revealed a scarred ulcer and 18Fluorodeoxyglucose(18FDG)positron emission tomography/computed tomography showed no abnormal FDG accumulation.The patient has been in complete remission for 68 mo after initial diagnosis.CONCLUSION To rule out MEITL,it is important to carefully perform histological examination when bleeding from a duodenal ulcer is observed.
文摘BACKGROUND Duodenal-type follicular lymphoma(D-FL)has been recognized as a rare entity that accounts for approximately 4%of primary gastrointestinal lymphomas.D-FL follows an indolent clinical course compared with common nodal FL and is generally considered to have a better prognosis.Therefore,the“watch and wait”approach is frequently adopted as the treatment method.Alternatively,there is an option to actively intervene in D-FL.However,the long-term outcomes of such cases are poorly understood.AIM To clarify the clinical outcomes after long-term follow-up in cases of D-FL with treatment intervention.METHODS We retrospectively analyzed patients who met the following criteria:the lesion was confirmed by endoscopy,the diagnosis of D-FL was confirmed histopathologically,and the patient was followed-up for more than 10 years after the intervention at our center.RESULTS We identified 5 cases of D-FL.Two patients showed a small amount of bone marrow involvement(Stage IV).Rituximab was used as a treatment for remission in all 5 patients.It was also used in combination with chemotherapy in 2 Stage IV patients as well as for maintenance treatment.Radiation therapy was performed in 2 cases,which was followed by complete remission(CR).Eventually,all 5 patients achieved CR and survived for more than 10 years.However,3 patients experienced recurrence.One patient achieved a second CR by retreatment,and in another case,the lesion showed spontaneous disappearance.The remaining patient had systemic widespread recurrence 13 years after the first CR.Biopsy results suggested that the FL lesions were transformed into diffuse large B-cell lymphoma.The patient died 4 years later despite receiving various chemotherapies.CONCLUSION In this study,the treatment for patients of D-FL in Stage IV was successful.In the future,criteria for how to treat“advanced”D-FL should be established based on additional cases.This study of patients with D-FL indicates that whole-body follow-up examinations should continue for a long time due to a fatal recurrence 13 years after reaching CR.
文摘This study investigated the effects of miRNA-155 on malignant biological characteristics of NK/T-cell lymphoma cell lines and the possible mechanism. The expression of miRNA-155 was detected in lymphoma cell lines from different sources (SNK-6, YTS, Jurkat and DOHH2) by real-time PCR. Lentiviral vectors (pLL3.7) that could overexpress or downexpress miRNA-155 were constructed. Recombinant lentiviral particles were prepared and purified, and their titers determined. The expression of miRNA-155 in the infected SNK-6 cells and the cell proliferation were detected by PCR and CCK-8, respectively. Flow cytometry was used to determine the apoptosis of infected SNK-6 cells. The target of miRNA155 was predicted from Targetscan website. The effect of miRNA155 on FOXO3a expression was examined by Western blotting. The results showed that among the human NK/T-cell lymphoma cell lines SNK-6, YTS, Jurkat and DOHH2, the expression of miRNA-155 was highest in SNK-6. The infection efficiency of the recombinant lentivirns in SNK-6 was more than 70% at multiplicity of infection (MOI) of 100. The expression of miRNA-155 was significantly increased in SNK-6 cells infected by lentivirus vectors with high expression of miRNA-155 (4 times higher than the control group), and profoundly decreased in those infected with lentivirnses with low expression of miRNA-155. The proliferation of letivirns-infected SNK-6 cells was decreased as the expression of miRNA-155 reduced. The apoptosis rate was increased with the reduction in the expression of miRNA-155. FOXO3a was found to be a possible target of miRNA155, as suggested by Targetscan website. Western blotting showed that the expression of FOXO3a was significantly elevated in SNK-6 cells with miRNA-155 inhibition. It was concluded that reduction in miRNA-155 expression can inhibit the proliferation of SNK-6 lymphoma cells and promote their apoptosis, which may be associated with regulation of FOXO3a gene.
基金Supported by The Scientific Research Foundation of the Ministry of Health,China,the Medical and Health Science Foundation,Zhejiang Province,China,No.WKJ-2009-2-021
文摘AIM:To study the diagnostic value of immunoglobulin heavy chain(IgH)and T-cell receptorγ (TCR-γ)gene monoclonal rearrangements in primary gastric lymphoma(PGL).METHODS:A total of 48 patients with suspected PGL at our hospital were prospectively enrolled in this study from January 2009 to December 2011.The patients were divided into three groups(a PGL group,a gastric linitis plastica group,and a benign gastric ulcer group)based on the pathological results(gastric mucosal specimens obtained by endoscopy or surgery)and follow-up.Endoscopic ultrasonography(EUS)and EUSguided biopsy were performed in all the patients.The tissue specimens were used for histopathological examination and for IgH and TCR-γ gene rearrangement polymerase chain reaction analyses.RESULTS:EUS and EUS-guided biopsy were successfully performed in all 48 patients.In the PGL group(n=21),monoclonal IgH gene rearrangements were detected in 14(66.7%)patients.A positive result for each set of primers was found in 12(57.1%),8(38.1%),and 4(19.0%)cases using FR1/JH,FR2/JH,and FR3/JH primers,respectively.Overall,12(75%)patients with mucosal-associated lymphoid tissue lymphoma(n=16)and 2(40%)patients with diffuse large B-cell lymphoma(n=5)were positive for monoclonal IgH gene rearrangements.No patients in the gastric linitis plastica group(n=17)and only one(10%)patient in the benign gastric ulcer group(n=10)were positive for a monoclonal IgH gene rearrangement.No TCRgene monoclonal rearrangements were detected.The sensitivity of monoclonal IgH gene rearrangements was 66.7%for a PGL diagnosis,and the specificity was96.4%.In the PGL group,8(100%)patients with stage IIE PGL(n=8)and 6(46.1%)patients with stage IE PGL(n=13)were positive for monoclonal IgH gene rearrangements.CONCLUSION:IgH gene rearrangements may be associated with PGL staging and may be useful for the diagnosis of PGL and for differentiating between PGL and gastric linitis plastica.
文摘T-cell/histiocyte-rich large B-cell lymphoma is uncommon in children population. There were few cases reported in the literature with wide range clinical presentations including advanced stage, and more involvement of liver, spleen and bone marrow. Head and neck lymphadenopathy tends to present in younger children. We report a case of 10-year-old boy who initially presented intermittent fever, headaches and neck lymphadenopathy. Subsequently, he developed diffuse lymphadenopathy and hepatosplenomegaly. T-cell/histiocyte-rich large B-cell lymphoma was diagnosed on a cervical lymph node biopsy. Cervical lymphadenopathy in this age group is most commonly reactive or nonmalignant processes. Lymphoma is much less frequent; mainly are non-Hodgkin lymphomas. However, a subset of large B-cell lymphoma called T-cell/histiocyte-rich B-cell lymphoma is rare in children.
基金supported by funds from the National Natural Science Foundation of China(Grant Nos.81830002,81830004,82070168,and 32070951)the Translational Research grant of NCRCH(Grant No.2020ZKZC04)National Key R&D Program of China(Grant No.2021YFA1100800)。
文摘Adoptive cellular immunotherapy with chimeric antigen receptor(CAR)T cells has emerged as a novel modality for treating relapsed and/or refractory B-cell non-Hodgkin lymphoma(B-NHL).With increasing approval of CAR T-cell products and advances in CAR T cell therapy,CAR T cells are expected to be used in a growing number of cases.However,CAR T-cell-associated toxicities can be severe or even fatal,thus compromising the survival benefit from this therapy.Standardizing and studying the clinical management of these toxicities are imperative.In contrast to other hematological malignancies,such as acute lymphoblastic leukemia and multiple myeloma,anti-CD19 CAR T-cell-associated toxicities in B-NHL have several distinctive features,most notably local cytokine-release syndrome(CRS).However,previously published guidelines have provided few specific recommendations for the grading and management of toxicities associated with CAR T-cell treatment for B-NHL.Consequently,we developed this consensus for the prevention,recognition,and management of these toxicities,on the basis of published literature regarding the management of anti-CD19 CAR T-cell-associated toxicities and the clinical experience of multiple Chinese institutions.This consensus refines a grading system and classification of CRS in B-NHL and corresponding measures for CRS management,and delineates comprehensive principles and exploratory recommendations for managing anti-CD19 CAR T-cell-associated toxicities in addition to CRS.
文摘Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital.The median age of these patients was 53.36±14.45 years.Female-male ratio was 1:1.2.The average course of disease was 10.82±3.37 years.All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51(40-74) Gy in an average of 24.55±5.57(20-40) fractions,5 fractions per week.Results The median follow-up time was 55.27±29.3(13-103) months.No severe acute or chronic side effects of irradiation were observed.Complete clinical response(CR) rate of the radiated sites was 54.5%(6/11),partial response(PR) rate was 36.4%(4/11),and the overall response rate(CR+PR) was 90.9%.One patient showed no response.Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.