BACKGROUND Chronic myeloid leukemia(CML)is a malignant hematologic malignancy that can progress to blast phase with a myeloid or lymphoid phenotype.Some patients with CML can also progress to blast crisis phase;howeve...BACKGROUND Chronic myeloid leukemia(CML)is a malignant hematologic malignancy that can progress to blast phase with a myeloid or lymphoid phenotype.Some patients with CML can also progress to blast crisis phase;however,the transformation of CML into Philadelphia-positive lymphoma is extremely rare.CASE SUMMARY We present a patient with CML who experienced a sudden transformation to anaplastic large-cell lymphoma(ALCL)after 7 mo of treatment with imatinib,during which she had achieved partial cytogenetic response as well as early molecular response.The patient noticed a mass in her left shoulder,the biopsy data of which were consistent with ALCL;moreover,her lymphoma cells exhibited BCR-ABL gene fusion.The patient was diagnosed with Philadelphia-positive ALCL that progressed from CML,and was thus treated with the second generation tyrosine kinase inhibitor nilotinib.Six months later,the mass had totally disappeared and the BCR-ABL fusion gene was undetectable in the peripheral blood.To our knowledge,this is the first patient known to have developed Philadelphia-positive ALCL transformed from CML.CONCLUSION Unexplained lymphadenopathy or an extramedullary mass in a patient with CML may warrant a biopsy and testing for BCR-ABL fusion.展开更多
An 82-year-old Japanese man visited our emergency unit complaining of dyspnea. Laboratory data showed 15% atypical lymphocytes in peripheral blood which expressed the T-cell phenotype. Chest/abdominal computed tomogra...An 82-year-old Japanese man visited our emergency unit complaining of dyspnea. Laboratory data showed 15% atypical lymphocytes in peripheral blood which expressed the T-cell phenotype. Chest/abdominal computed tomography depicted hepatosplenomegaly and swelling of systemic lymph nodes. The patient died of advanced respiratory failure 5 d after the first occurrence of his dyspnea. At autopsy, the pathological features revealed a diffuse infiltration of large atypical lymphocytes to systemic organs including the spleen and lung. In immunohistochemical staining, these cells expressed CD30, TIA-1, anaplastic lymphoma kinase(ALK), CD5 and CD3. An advanced surface molecule analysis revealed a lack of CD54(intercellular cell adhesion molecule-1) and CD56(neural cell adhesion molecule). We observed the proliferation and infiltration of these lymphoma cells specifically at the intravascular lesions similar to intravascular lymphoma(IVL). T-cell IVL is not established as an independent clinical entity in the World Health Organization classification, and our patient's ALK-positive T-IVL in lung appears to be the first reported case.展开更多
BACKGROUND Anaplastic lymphoma kinase(ALK)-positive large B-cell lymphoma(LBCL)is an aggressive and rare variant of diffuse LBCL.Herein,we report an uncommon case of stage IE extranodal ALK-positive LBCL initially ori...BACKGROUND Anaplastic lymphoma kinase(ALK)-positive large B-cell lymphoma(LBCL)is an aggressive and rare variant of diffuse LBCL.Herein,we report an uncommon case of stage IE extranodal ALK-positive LBCL initially originating in the bulbar con-junctiva.CASE SUMMARY A 63-year-old woman presented with a mass in the left bulbar conjunctiva that had persisted for six months,accompanied by swelling and pain that had per-sisted for 3 d.Eye examination revealed an 8 mm slightly elevated pink mass in the lower conjunctival sac of the left eye.Microscopically,the tumor was com-posed of large immunoblastic and plasmablastic large lymphoid cells with scattered anaplastic or multinucleated large cells.Immunophenotypically,the neoplastic cells were positive for ALK,CD10,CD138,Kappa,MUM1,BOB.1,OCT-2,CD4,CD45,EMA,CD79a,CD38,and AE1/AE3,and negative for CD20,PAX5,Lambda,BCL6,CD30 and all other T-cell antigens.The results of gene rearrangement tests showed monoclonal IGH/IGK/IGL and TCRD rearran-gements.Fluorescence in situ hybridization studies did not reveal any BCL2,BCL6 or MYC rearrangements.Furthermore,Epstein-Barr virus was not detected by in situ hybridization in the lesions.Based on the histopathological and imaging examinations,the neoplasm was classified as stage IE ALK-positive LBCL.No further treatments were administered.At the 6,15,and 21 mo postoperative follow-up visits,the patient was in good condition,without obvious discomfort.This case represents the first example of primary extranodal ALK-positive LBCL presenting as a bulbar conjunctival mass,which is extremely rare and shares morphological and immunohistochemical features with a variety of other neo-plasms that can result in misdiagnosis.CONCLUSION Awareness of the condition presented in this case report is necessary for early and accurate diagnosis and appropriate treatment.展开更多
BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other derma...BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.展开更多
Anaplastic large-cell lymphoma(ALCL) is characterized by frequently presenting adverse factors at diagnosis.Many groups believed aggressive treatment strategies such as autologous stem cell transplantation brought sur...Anaplastic large-cell lymphoma(ALCL) is characterized by frequently presenting adverse factors at diagnosis.Many groups believed aggressive treatment strategies such as autologous stem cell transplantation brought survival benefit for ALCL patients.However,few compared these approaches with conventional chemotherapy to validate their superiority.Here,we report a study comparing the efficacy of peripheral blood stem cell transplantation(PBSCT) and conventional chemotherapy on ALCL.A total of 64 patients with primary systemic ALCL were studied retrospectively.The median follow-up period was 51 months(range,1-167 months).For 48 patients undergoing conventional chemotherapy only,the 4-year event-free survival(EFS) and overall survival(OS) rates were 70.7% and 88.3%,respectively.Altogether,16 patients underwent PBSCT,including 11 at first remission(CR1/PR1),3 at second remission,and 2 with disease progression during first-line chemotherapy.The 4-year EFS and OS rates for patients underwent PBSCT at first remission were 81.8% and 90.9%,respectively.Compared with conventional chemotherapy,PBSCT did not show superiority either in EFS(P = 0.240) or in OS(P = 0.580) when applied at first remission.Univariate analysis showed that patients with B symptoms(P = 0.001),stage III/IV disease(P = 0.008),bulky disease(P = 0.075),negative anaplastic lymphoma kinase(ALK) expression(P = 0.059),and age ≤ 60 years(P = 0.054) had lower EFS.Furthermore,PBSCT significantly improved EFS in patients with B symptoms(100% vs.50.8%,P = 0.027) or bulky disease(100% vs.52.8%,P = 0.045) when applied as an up-front strategy.Based on these results,we conclude that,for patients with specific adverse factors such as B symptoms and bulky disease,PBSCT was superior to conventional chemotherapy in terms of EFS.展开更多
BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)differs from systemic anaplastic large cell lymphoma(sALCL)in cell biological behavior,clinical features,treatment,and outcome.PC-ALCL has been repor...BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)differs from systemic anaplastic large cell lymphoma(sALCL)in cell biological behavior,clinical features,treatment,and outcome.PC-ALCL has been reported to rarely transition into sALCL,but the underlying mechanism is not clear.Here we report such a case with certain characteristics that shed light on this.CASE SUMMARY Herein,we report a 43-year-old male with symptoms of a skin nodule and histologically confirmed PC-ALCL with high expression of Ki-67.After three months of observation,two skin nodules re-appeared with muscle layer involvement and was histologically confirmed as sALCL.Seventeen months after receiving six cycles of CHOP regimen,the patient had pain in the chest and back,cough,shortness of breath,and night sweats.This was confirmed as relapse of sALCL by immunohistochemistry and several organs,such as the lung were involved as shown by positron emission tomography/computed tomography.After four cycles of DICE plus chidamide regimens followed by auto-hematopoietic stem cell transplantation(ASCT),complete remission(CR)duration was achieved for twelve months while the patient was on maintenance with chidamide(20 mg)pills.CONCLUSION This case had significantly high expression of Ki-67 when diagnosed as PC-ALCL initially and then transitioned into sALCL,which is rare.Auto-ASCT combined with demethylation drugs effectively maintained CR and prolonged progression free survival.展开更多
BACKGROUND The development of anaplastic lymphoma kinase(ALK)-tyrosine kinase inhibitors(TKIs)has remarkably improved the prognosis of patients with ALK-positive advanced non-small cell lung cancer(NSCLC).Alectinib,th...BACKGROUND The development of anaplastic lymphoma kinase(ALK)-tyrosine kinase inhibitors(TKIs)has remarkably improved the prognosis of patients with ALK-positive advanced non-small cell lung cancer(NSCLC).Alectinib,the second-generation ALK-TKI,has been approved as first-line treatment for advanced or metastatic NSCLC patients with ALK rearrangement.Neoadjuvant therapy can achieve tumor downstaging and eradicate occult lesions in patients with potentially resectable disease.Whether neoadjuvant alectinib can be a conversion therapy in ALK-positive advanced NSCLC patients remains unclear.CASE SUMMARY A 41-year-old man was pathologically diagnosed with locally advanced ALKpositive stage IIIB NSCLC.Alectinib was prescribed to induce tumor downstaging and facilitate the subsequent surgical resection.The tumor was successfully downstaged and pathological complete response was achieved.Left upper lobectomy with mediastinal lymphadenectomy was performed after tumor downstaging.The patient has continued to receive alectinib as adjuvant therapy during postoperative follow-up with a recurrence-free survival of 29 mo as of writing this report.CONCLUSION This case sheds light on the feasibility and safety of alectinib as a neoadjuvant treatment for stage IIIB NSCLC patients with ALK rearrangement.Its efficacy needs to be validated in prospective clinical trials.展开更多
BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare t...BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.展开更多
BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and it...BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and its reported incidence in Asia is 1 or less per 100000 people per year.The co-occurrence of sarcoidosis and ALK-positive NSCLC is rare,and ALK-positive lung cancer is likely to spread quickly.Therefore,the co-occurrence of sarcoidosis is more easily misdiagnosed as metastatic lung cancer by radiological examination.CASE SUMMARY A 50-year-old man had a nodule in the left superior lobe,many small nodules in left superior and right lungs,and enlarged bilateral hilar,mediastinal,and right supraclavicular lymph nodes.Computed tomography-guided pulmonary biopsy of the nodule in the left superior lobe revealed echinoderm microtubuleassociated protein-like 4 gene-ALK positive NSCLC with concomitant noncaseating granuloma.This patient was treated with crizotinib.Thirty days later,a chest computed tomography scan revealed a dramatic decrease in the size of the left superior lobe nodule;however,the lesions in the right lung progressed.The right supraclavicular lymph nodes showed granulomas,and no tumor cells were identified in the specimens. The angiotensin-converting enzyme level was high.After 1 wk of methylprednisolone treatment, a significant response of all lesionswas revealed. Following radical resection of the lung cancer, noncaseatinggranulomas were observed in both lung tissues and lymph nodes, which resultedin a diagnosis of echinoderm microtubule-associated protein-like 4-ALK positiveNSCLC accompanied with sarcoidosis.CONCLUSIONOur experience illustrates that pathological evidence is needed to confirmmetastatic disease, especially when some suspected metastatic lesions arenegative for malignancy.展开更多
We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, ...We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, weight loss and fatigue. His laboratory tests showed anemia and a great elevation of ESR, LDH, and β2 microglobulin. In CT and MRI scan, a soft tissue mass in the pancreas was observed. A repeated endoscopy after his admission revealed an ulcerated mass-like deformity of the duodenal bulb. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant pancreatic mass extending to the adjacent organs. Duodenal and surgical biopsies identified an ALCL of T-cell lineage, ALK-. The patient died in the Intensive Care Unit due to hemodynamic instability.Our case is the first one indicating that primary pancreatic lymphoma should be suspected in a patient with pancreatic mass and elevated serum LDH and β2 microglobulin.展开更多
BACKGROUND Anaplastic large cell lymphoma(ALCL)is a type of T-cell lymphoma that can be divided into two categories:anaplastic lymphoma kinase-positive(ALK+)and ALK-negative.Gastrointestinal ALK+ALCL is rare.Multiple ...BACKGROUND Anaplastic large cell lymphoma(ALCL)is a type of T-cell lymphoma that can be divided into two categories:anaplastic lymphoma kinase-positive(ALK+)and ALK-negative.Gastrointestinal ALK+ALCL is rare.Multiple lymphomatous polyposis(MLP)is thought to be a representative form of gastrointestinal lesion in mantle cell lymphoma,and T-cell lymphomas seldom show this feature.Here,we report the first known case of ALK+ALCL with gastroduodenal involvement to present with MLP.CASE SUMMARY The patient was a 43-year-old man who was complained of a mass in the left inguinal area and was performed open biopsy.ALK+ALCL was diagnosed pathologically.Computed tomography scan demonstrated multiple lymph node lesions in the abdomen-pelvis/inguinal region,and scattered nodular lesions in both lung fields.He did not complain of gastrointestinal symptoms.While,esophagogastroduodenoscopy identified MLP lesions from the antrum of the stomach to the descending portion of the duodenum and mild thickened folds on the corpus of the stomach,and biopsy showed invasion of ALK+ALCL.We treated this patient with six cycles of CHOEP(Cyclophosphamide,Doxorubicin,Vincristine,Etoposide,and Prednisone)chemotherapy.At the conclusion of treatment,there was complete remission.Numerous white scars were found on the stomach,endoscopically consistent with a remission image of lymphoma.The endoscopic features of this case were thought to be similar to those of MCL.CONCLUSION The macroscopic/endoscopic features of gastrointestinal ALK+ALCL may be more similar to those of B-cell lymphomas rather than T-cell lymphomas.展开更多
BACKGROUND Anaplastic large cell lymphoma(ALCL)is a rare and heterogeneous malignant tumor,which is classied as anaplastic lymphoma kinase(ALK)positive ALCL and ALK-ALCL.Many patients are diagnosed with ALCL at the s...BACKGROUND Anaplastic large cell lymphoma(ALCL)is a rare and heterogeneous malignant tumor,which is classied as anaplastic lymphoma kinase(ALK)positive ALCL and ALK-ALCL.Many patients are diagnosed with ALCL at the stage of bone marrow involvement.However,ALCL patients with clinical manifestations consistent with acute leukemia are relatively rare.CASE SUMMARY In this report,the patient did not receive appropriate diagnosis and treatment despite a two-year history of lymph node enlargement.Hereafter,she was admitted for B symptoms and was diagnosed as ALK-ALCL by lymph node biopsy.Then,the disease progressed to leukemia without any treatment after 2 mo.The proportion of lymphoma cells in bone marrow was as high as 96%,and the proportion of peripheral blood was 84%.She also had clinical manifestations similar to acute leukemia.After completion of chemotherapy,she developed granulocytopenia and fever and died from septicemia.CONCLUSION ALCL with leukemic presentation is a late manifestation of lymphoma with low chemotherapy tolerance and poor prognosis.展开更多
INTRODUCTION Non-small cell lung cancer(NSCLC)is a common malignant disease with an extremely poor prognosis.Lung cancer has been reported to metastasize to the eye in 0.2%to7%of patients based on clinical studies,a...INTRODUCTION Non-small cell lung cancer(NSCLC)is a common malignant disease with an extremely poor prognosis.Lung cancer has been reported to metastasize to the eye in 0.2%to7%of patients based on clinical studies,and in 6%to 7%of patients based on postmortem histopathologic studies.展开更多
Primary lymphoma involving the breast accounts for only 0.04-0.5%of malignant breast tumors,constituting 1-2%of all non-Hodgkin lymphomas(NHL).Here,we report a case of breast ALK-positive ALCL arising in a 21-year-old...Primary lymphoma involving the breast accounts for only 0.04-0.5%of malignant breast tumors,constituting 1-2%of all non-Hodgkin lymphomas(NHL).Here,we report a case of breast ALK-positive ALCL arising in a 21-year-old woman in pregnancy with single abscess as the first clinical manifestation.展开更多
BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma ...BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase(ALK)-positive and ALK-negative groups.ALCL rarely presents in the gastrointestinal tract.CASE SUMMARY A 54-year-old male was admitted to the department of gastroenterology for abdominal pain.He presented with lower abdominal pain,diarrhea and recurrent oral and penile ulcers.He was misdiagnosed with Behcet's disease and treated with prednisone.But after one month,he was hospitalized in another hospital for reexamination.This time,the lesion on the penis was biopsied for histological examination.The final pathological diagnosis was ALCL,ALKnegative.The patient was treated with cyclophosphamide,doxorubicin,vincristine,prednisolone chemotherapy.However,he died within one month.CONCLUSION Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment.Repeated biopsy is the most important for early diagnosis and treatment.展开更多
Introduction: The rearrangement of the anaplastic lymphoma kinase(ALK) gene accounts for approximately 1%–6% of lung adenocarcinoma cases and deines a molecular subgroup of tumors characterized by clinical sensitivit...Introduction: The rearrangement of the anaplastic lymphoma kinase(ALK) gene accounts for approximately 1%–6% of lung adenocarcinoma cases and deines a molecular subgroup of tumors characterized by clinical sensitivity to ALK inhibitors such as crizotinib. This study aimed to identify the relationship between ALK rearrangement and the clinico?pathologic characteristics of non?small cell lung cancer(NSCLC) and to analyze the therapeutic responses of crizotinib and conventional chemotherapy to ALK rearrangement in NSCLC patients.Methods: A total of 487 lung cancer patients who underwent testing for ALK rearrangement in our department were included in this study. ALK rearrangement was examined by using fluorescence in situ hybridization(FISH) assay.Results: Among the 487 patients, 44(9.0%) were diagnosed with ALK rearrangement by using FISH assay. In 123 patients with adenocarcinoma who were non?smokers and of a young age(≤58 years old), the frequency of ALK rearrangement was 20.3%(25/123). Short overall survival(OS) was associated with non?adenocarcinoma tumor type(P = 0.006), poorly diferentiated tumors(P al growth factor rece= 0.001), advanced?stage tumors(P < 0.001), smoking history(P ptor(EGFR)(P = 0.008), and wild?type epidermrter time to cancer p= 0.008). Moreover, patients with poorly diferentiated and advanced?stage tumors had a shorogression compared with those with well diferentiated(P = 0.023) and early?stage tumors(P = 0.001), respectively.Conclusions: ALK?rearranged NSCLC tends to occur in younger individuals who are either non?smokers or light smokers with adenocarcinoma. Patients with ALK rearrangement might beneit from ALK inhibitor therapy.展开更多
Aberrant forms of the anaplastic lymphoma kinase(ALK) are involved in the pathogenesis of several types of cancer, including anaplastic large cell lymphoma, non-small-cell lung cancer(NSCLC), inflammatory myofibroblas...Aberrant forms of the anaplastic lymphoma kinase(ALK) are involved in the pathogenesis of several types of cancer, including anaplastic large cell lymphoma, non-small-cell lung cancer(NSCLC), inflammatory myofibroblastic tumors, colorectal cancer, neuroblastoma and others. In general, the ALK catalytic domain is rearranged and fused to a dimerization domain encoded by an unrelated gene. Less frequently, full-length ALK is activated by point mutations. The common theme is unregulated firing of ALK downstream signalling, leading to uncontrolled cell division and increased cell survival. ALK-driven tumors can be treated with Crizotinib, an orally available dual ALK/MET inhibitor, currently approved for advanced ALK-positive NSCLCs. Crizotinibtreated patients achieve high response rates, with an excellent toxicity profile. However, drug-resistant disease often develops, particularly in NSCLC patients. The processes leading to drug resistance include both ALKdependent(point mutations or gene amplification), as well as ALK-independent mechanisms, which are here briefly discussed. Recently, Ceritinib has been approved for Crizotinib-refractory NSCLC, further extending patients' survival, but resistance again emerged. Novel ALK kinase inhibitors are currently under clinical development, showing great promise for improved efficacy in drugresistance disease. It is opinion of the author that drugresistance is likely to arise under any treatment, due to intrinsic heterogeneity and adaptability of cancer. To prevent or delay this phenomenon, we need to treat less advanced disease, with drugs that are rapidly effective in order not to allow enough time for tumor evolution, and we want to have more and more drugs with nonoverlapping resistance profiles, for subsequent lines of targeted therapy. Finally, the use of drug combinations may exponentially decrease the chances of resistance.展开更多
Kawasaki disease (KD) is an acute febrile systemic vasculitis occurring predominantly in young children less than 5 years of age. Although imperfectly known, the aetiopathogenesis of KD would be secondary to immunolog...Kawasaki disease (KD) is an acute febrile systemic vasculitis occurring predominantly in young children less than 5 years of age. Although imperfectly known, the aetiopathogenesis of KD would be secondary to immunological abnormalities that could constitute a favourable terrain for neoplasms. We report on a case in a 2-year-old girl who presented clinical manifestations compatible with Kawasaki disease complicated by coronary aneurysm. Aetiopathological investigations revealed M. pneumoniae infection as specific IgM were present in the serum (Elisa). The patient was initially treated by intravenous immunoglobulins (IVIG) and aspirin to anti-inflammatory dose. Following a few days of desquamation, resolution of the symptomatology occurred. Four weeks later she developed disseminated tumorous syndrome. Lymph node biopsy revealed massive infiltration by large cells lymphomatous proliferation. Histologic and immunophenotypic findings were characteristic of ALK-1+ anaplastic large cell lymphoma. Four weeks later, the patient died from a severe nosocomial infection complicated by septic shock. Our observation is the first cases describing the association between anaplastic large cell lymphoma, KD and M. pneumoniae. Immunologic disorder due to KD and M. pneumoniae infection may play probably a central role for malignancy.展开更多
BACKGROUND Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin’s lymphoma (NHL). ALCL is rare as a bone lesion and in pregnancy. CASE SUMMARY We present the first case of anaplastic lymphoma kinase (ALK)+ ...BACKGROUND Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin’s lymphoma (NHL). ALCL is rare as a bone lesion and in pregnancy. CASE SUMMARY We present the first case of anaplastic lymphoma kinase (ALK)+ ALCL of the thoracic spine during pregnancy. A 25-year-old pregnant woman was presented to us at 24 wk’ gestation with severe back pain and weakness in the left lower limb. Imaging examination showed lesions at T10 and T11. She underwent posterior pedicle screw fixation and vertebroplasty. Pathological examination showed ALK+ ALCL. The patient chose to have therapeutic abortion after surgery and received chemotherapy in the hematology department. She now remains disease free with no neurological deficit after 30 mo’ follow-up. CONCLUSION ALK+ ALCL with the thoracic spine involvement is uncommon, especially in pregnancy. Many symptoms can be misunderstood during pregnancy;therefore, when a pregnant patient has persistent back pain or lower limb neurological symptoms, imaging examinations should be performed.展开更多
Introduction: Anaplastic large-cell lymphoma occurring in the nasal cavity is a rare disease. The latest World Health Organization (WHO) Classification recognizes three distinct subtypes: primary systemic anaplastic l...Introduction: Anaplastic large-cell lymphoma occurring in the nasal cavity is a rare disease. The latest World Health Organization (WHO) Classification recognizes three distinct subtypes: primary systemic anaplastic lymphoma kinase positive been our case, primary systemic anaplastic lymphoma kinase negative and primary cutaneous types. Through this case study, we focus on the clinical presentation, treatment and prognostic characteristics of this pathology.Case Presentation: We report the case of a patient aged 32 years, who presented for seven months a runny nose associated with swelling of the face on the left side, without peripheral lymphadenopathy or general signs. A Blondeau scanner objectified a total filling of the frontal and left maxillary sinus, and a filling of the left nasal cavity. Complete resection of the tumor was performed. Histological examination was in favor of anaplastic large T-cell lymphoma anaplastic lymphoma kinase positive. The patient was stage IE according to Ann Arbor classification, with an International Prognostic Index estimated at one. Thus, the patient received six cycles of CHOP chemotherapy. Currently, he is in good loco-regional control with a decline of three months.Conclusion: The rarity of this case lies partly in the lymphomatous localization in the nasal cavity, and secondly in the anaplastic histology. It poses a diagnostic problem. So, we conclude that in case of any symptom of nasal cavities, it is necessary to explore and possibly biopsy if tumor, before surgery, because lymphomas are chemosensitive disease.展开更多
基金Supported by the Jiangxi“5511”Science and Technology Innovation Talent Project,No.20171BCB18003。
文摘BACKGROUND Chronic myeloid leukemia(CML)is a malignant hematologic malignancy that can progress to blast phase with a myeloid or lymphoid phenotype.Some patients with CML can also progress to blast crisis phase;however,the transformation of CML into Philadelphia-positive lymphoma is extremely rare.CASE SUMMARY We present a patient with CML who experienced a sudden transformation to anaplastic large-cell lymphoma(ALCL)after 7 mo of treatment with imatinib,during which she had achieved partial cytogenetic response as well as early molecular response.The patient noticed a mass in her left shoulder,the biopsy data of which were consistent with ALCL;moreover,her lymphoma cells exhibited BCR-ABL gene fusion.The patient was diagnosed with Philadelphia-positive ALCL that progressed from CML,and was thus treated with the second generation tyrosine kinase inhibitor nilotinib.Six months later,the mass had totally disappeared and the BCR-ABL fusion gene was undetectable in the peripheral blood.To our knowledge,this is the first patient known to have developed Philadelphia-positive ALCL transformed from CML.CONCLUSION Unexplained lymphadenopathy or an extramedullary mass in a patient with CML may warrant a biopsy and testing for BCR-ABL fusion.
文摘An 82-year-old Japanese man visited our emergency unit complaining of dyspnea. Laboratory data showed 15% atypical lymphocytes in peripheral blood which expressed the T-cell phenotype. Chest/abdominal computed tomography depicted hepatosplenomegaly and swelling of systemic lymph nodes. The patient died of advanced respiratory failure 5 d after the first occurrence of his dyspnea. At autopsy, the pathological features revealed a diffuse infiltration of large atypical lymphocytes to systemic organs including the spleen and lung. In immunohistochemical staining, these cells expressed CD30, TIA-1, anaplastic lymphoma kinase(ALK), CD5 and CD3. An advanced surface molecule analysis revealed a lack of CD54(intercellular cell adhesion molecule-1) and CD56(neural cell adhesion molecule). We observed the proliferation and infiltration of these lymphoma cells specifically at the intravascular lesions similar to intravascular lymphoma(IVL). T-cell IVL is not established as an independent clinical entity in the World Health Organization classification, and our patient's ALK-positive T-IVL in lung appears to be the first reported case.
文摘BACKGROUND Anaplastic lymphoma kinase(ALK)-positive large B-cell lymphoma(LBCL)is an aggressive and rare variant of diffuse LBCL.Herein,we report an uncommon case of stage IE extranodal ALK-positive LBCL initially originating in the bulbar con-junctiva.CASE SUMMARY A 63-year-old woman presented with a mass in the left bulbar conjunctiva that had persisted for six months,accompanied by swelling and pain that had per-sisted for 3 d.Eye examination revealed an 8 mm slightly elevated pink mass in the lower conjunctival sac of the left eye.Microscopically,the tumor was com-posed of large immunoblastic and plasmablastic large lymphoid cells with scattered anaplastic or multinucleated large cells.Immunophenotypically,the neoplastic cells were positive for ALK,CD10,CD138,Kappa,MUM1,BOB.1,OCT-2,CD4,CD45,EMA,CD79a,CD38,and AE1/AE3,and negative for CD20,PAX5,Lambda,BCL6,CD30 and all other T-cell antigens.The results of gene rearrangement tests showed monoclonal IGH/IGK/IGL and TCRD rearran-gements.Fluorescence in situ hybridization studies did not reveal any BCL2,BCL6 or MYC rearrangements.Furthermore,Epstein-Barr virus was not detected by in situ hybridization in the lesions.Based on the histopathological and imaging examinations,the neoplasm was classified as stage IE ALK-positive LBCL.No further treatments were administered.At the 6,15,and 21 mo postoperative follow-up visits,the patient was in good condition,without obvious discomfort.This case represents the first example of primary extranodal ALK-positive LBCL presenting as a bulbar conjunctival mass,which is extremely rare and shares morphological and immunohistochemical features with a variety of other neo-plasms that can result in misdiagnosis.CONCLUSION Awareness of the condition presented in this case report is necessary for early and accurate diagnosis and appropriate treatment.
文摘BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.
文摘Anaplastic large-cell lymphoma(ALCL) is characterized by frequently presenting adverse factors at diagnosis.Many groups believed aggressive treatment strategies such as autologous stem cell transplantation brought survival benefit for ALCL patients.However,few compared these approaches with conventional chemotherapy to validate their superiority.Here,we report a study comparing the efficacy of peripheral blood stem cell transplantation(PBSCT) and conventional chemotherapy on ALCL.A total of 64 patients with primary systemic ALCL were studied retrospectively.The median follow-up period was 51 months(range,1-167 months).For 48 patients undergoing conventional chemotherapy only,the 4-year event-free survival(EFS) and overall survival(OS) rates were 70.7% and 88.3%,respectively.Altogether,16 patients underwent PBSCT,including 11 at first remission(CR1/PR1),3 at second remission,and 2 with disease progression during first-line chemotherapy.The 4-year EFS and OS rates for patients underwent PBSCT at first remission were 81.8% and 90.9%,respectively.Compared with conventional chemotherapy,PBSCT did not show superiority either in EFS(P = 0.240) or in OS(P = 0.580) when applied at first remission.Univariate analysis showed that patients with B symptoms(P = 0.001),stage III/IV disease(P = 0.008),bulky disease(P = 0.075),negative anaplastic lymphoma kinase(ALK) expression(P = 0.059),and age ≤ 60 years(P = 0.054) had lower EFS.Furthermore,PBSCT significantly improved EFS in patients with B symptoms(100% vs.50.8%,P = 0.027) or bulky disease(100% vs.52.8%,P = 0.045) when applied as an up-front strategy.Based on these results,we conclude that,for patients with specific adverse factors such as B symptoms and bulky disease,PBSCT was superior to conventional chemotherapy in terms of EFS.
文摘BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)differs from systemic anaplastic large cell lymphoma(sALCL)in cell biological behavior,clinical features,treatment,and outcome.PC-ALCL has been reported to rarely transition into sALCL,but the underlying mechanism is not clear.Here we report such a case with certain characteristics that shed light on this.CASE SUMMARY Herein,we report a 43-year-old male with symptoms of a skin nodule and histologically confirmed PC-ALCL with high expression of Ki-67.After three months of observation,two skin nodules re-appeared with muscle layer involvement and was histologically confirmed as sALCL.Seventeen months after receiving six cycles of CHOP regimen,the patient had pain in the chest and back,cough,shortness of breath,and night sweats.This was confirmed as relapse of sALCL by immunohistochemistry and several organs,such as the lung were involved as shown by positron emission tomography/computed tomography.After four cycles of DICE plus chidamide regimens followed by auto-hematopoietic stem cell transplantation(ASCT),complete remission(CR)duration was achieved for twelve months while the patient was on maintenance with chidamide(20 mg)pills.CONCLUSION This case had significantly high expression of Ki-67 when diagnosed as PC-ALCL initially and then transitioned into sALCL,which is rare.Auto-ASCT combined with demethylation drugs effectively maintained CR and prolonged progression free survival.
文摘BACKGROUND The development of anaplastic lymphoma kinase(ALK)-tyrosine kinase inhibitors(TKIs)has remarkably improved the prognosis of patients with ALK-positive advanced non-small cell lung cancer(NSCLC).Alectinib,the second-generation ALK-TKI,has been approved as first-line treatment for advanced or metastatic NSCLC patients with ALK rearrangement.Neoadjuvant therapy can achieve tumor downstaging and eradicate occult lesions in patients with potentially resectable disease.Whether neoadjuvant alectinib can be a conversion therapy in ALK-positive advanced NSCLC patients remains unclear.CASE SUMMARY A 41-year-old man was pathologically diagnosed with locally advanced ALKpositive stage IIIB NSCLC.Alectinib was prescribed to induce tumor downstaging and facilitate the subsequent surgical resection.The tumor was successfully downstaged and pathological complete response was achieved.Left upper lobectomy with mediastinal lymphadenectomy was performed after tumor downstaging.The patient has continued to receive alectinib as adjuvant therapy during postoperative follow-up with a recurrence-free survival of 29 mo as of writing this report.CONCLUSION This case sheds light on the feasibility and safety of alectinib as a neoadjuvant treatment for stage IIIB NSCLC patients with ALK rearrangement.Its efficacy needs to be validated in prospective clinical trials.
基金Supported by National Science and Technology Major Subproject of China,No.2018ZX10302205-002Chinese Foundation for Hepatitis Prevention and Control-Tianqing Liver Disease Research Fund Subject,No.TQGB2020168.
文摘BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.
基金The Zhejiang Provincial Natural Science Foundation of China,No.LQ17H160005.
文摘BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and its reported incidence in Asia is 1 or less per 100000 people per year.The co-occurrence of sarcoidosis and ALK-positive NSCLC is rare,and ALK-positive lung cancer is likely to spread quickly.Therefore,the co-occurrence of sarcoidosis is more easily misdiagnosed as metastatic lung cancer by radiological examination.CASE SUMMARY A 50-year-old man had a nodule in the left superior lobe,many small nodules in left superior and right lungs,and enlarged bilateral hilar,mediastinal,and right supraclavicular lymph nodes.Computed tomography-guided pulmonary biopsy of the nodule in the left superior lobe revealed echinoderm microtubuleassociated protein-like 4 gene-ALK positive NSCLC with concomitant noncaseating granuloma.This patient was treated with crizotinib.Thirty days later,a chest computed tomography scan revealed a dramatic decrease in the size of the left superior lobe nodule;however,the lesions in the right lung progressed.The right supraclavicular lymph nodes showed granulomas,and no tumor cells were identified in the specimens. The angiotensin-converting enzyme level was high.After 1 wk of methylprednisolone treatment, a significant response of all lesionswas revealed. Following radical resection of the lung cancer, noncaseatinggranulomas were observed in both lung tissues and lymph nodes, which resultedin a diagnosis of echinoderm microtubule-associated protein-like 4-ALK positiveNSCLC accompanied with sarcoidosis.CONCLUSIONOur experience illustrates that pathological evidence is needed to confirmmetastatic disease, especially when some suspected metastatic lesions arenegative for malignancy.
文摘We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, weight loss and fatigue. His laboratory tests showed anemia and a great elevation of ESR, LDH, and β2 microglobulin. In CT and MRI scan, a soft tissue mass in the pancreas was observed. A repeated endoscopy after his admission revealed an ulcerated mass-like deformity of the duodenal bulb. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant pancreatic mass extending to the adjacent organs. Duodenal and surgical biopsies identified an ALCL of T-cell lineage, ALK-. The patient died in the Intensive Care Unit due to hemodynamic instability.Our case is the first one indicating that primary pancreatic lymphoma should be suspected in a patient with pancreatic mass and elevated serum LDH and β2 microglobulin.
文摘BACKGROUND Anaplastic large cell lymphoma(ALCL)is a type of T-cell lymphoma that can be divided into two categories:anaplastic lymphoma kinase-positive(ALK+)and ALK-negative.Gastrointestinal ALK+ALCL is rare.Multiple lymphomatous polyposis(MLP)is thought to be a representative form of gastrointestinal lesion in mantle cell lymphoma,and T-cell lymphomas seldom show this feature.Here,we report the first known case of ALK+ALCL with gastroduodenal involvement to present with MLP.CASE SUMMARY The patient was a 43-year-old man who was complained of a mass in the left inguinal area and was performed open biopsy.ALK+ALCL was diagnosed pathologically.Computed tomography scan demonstrated multiple lymph node lesions in the abdomen-pelvis/inguinal region,and scattered nodular lesions in both lung fields.He did not complain of gastrointestinal symptoms.While,esophagogastroduodenoscopy identified MLP lesions from the antrum of the stomach to the descending portion of the duodenum and mild thickened folds on the corpus of the stomach,and biopsy showed invasion of ALK+ALCL.We treated this patient with six cycles of CHOEP(Cyclophosphamide,Doxorubicin,Vincristine,Etoposide,and Prednisone)chemotherapy.At the conclusion of treatment,there was complete remission.Numerous white scars were found on the stomach,endoscopically consistent with a remission image of lymphoma.The endoscopic features of this case were thought to be similar to those of MCL.CONCLUSION The macroscopic/endoscopic features of gastrointestinal ALK+ALCL may be more similar to those of B-cell lymphomas rather than T-cell lymphomas.
基金Supported by Joint Project of Natural Science Foundation of Shandong Province,No.ZR2015HL037.
文摘BACKGROUND Anaplastic large cell lymphoma(ALCL)is a rare and heterogeneous malignant tumor,which is classied as anaplastic lymphoma kinase(ALK)positive ALCL and ALK-ALCL.Many patients are diagnosed with ALCL at the stage of bone marrow involvement.However,ALCL patients with clinical manifestations consistent with acute leukemia are relatively rare.CASE SUMMARY In this report,the patient did not receive appropriate diagnosis and treatment despite a two-year history of lymph node enlargement.Hereafter,she was admitted for B symptoms and was diagnosed as ALK-ALCL by lymph node biopsy.Then,the disease progressed to leukemia without any treatment after 2 mo.The proportion of lymphoma cells in bone marrow was as high as 96%,and the proportion of peripheral blood was 84%.She also had clinical manifestations similar to acute leukemia.After completion of chemotherapy,she developed granulocytopenia and fever and died from septicemia.CONCLUSION ALCL with leukemic presentation is a late manifestation of lymphoma with low chemotherapy tolerance and poor prognosis.
文摘INTRODUCTION Non-small cell lung cancer(NSCLC)is a common malignant disease with an extremely poor prognosis.Lung cancer has been reported to metastasize to the eye in 0.2%to7%of patients based on clinical studies,and in 6%to 7%of patients based on postmortem histopathologic studies.
文摘Primary lymphoma involving the breast accounts for only 0.04-0.5%of malignant breast tumors,constituting 1-2%of all non-Hodgkin lymphomas(NHL).Here,we report a case of breast ALK-positive ALCL arising in a 21-year-old woman in pregnancy with single abscess as the first clinical manifestation.
基金Supported by National Natural Science Foundation of China,No.U1802282,No.81660100,and No.81670501MDT Innovation Team to Digestive Diseases of S&T Department of Yunnan Province,No.2017HC005Digestive Diseases S&T Innovation Team of Kunming Medical University,No.CXTD201611
文摘BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase(ALK)-positive and ALK-negative groups.ALCL rarely presents in the gastrointestinal tract.CASE SUMMARY A 54-year-old male was admitted to the department of gastroenterology for abdominal pain.He presented with lower abdominal pain,diarrhea and recurrent oral and penile ulcers.He was misdiagnosed with Behcet's disease and treated with prednisone.But after one month,he was hospitalized in another hospital for reexamination.This time,the lesion on the penis was biopsied for histological examination.The final pathological diagnosis was ALCL,ALKnegative.The patient was treated with cyclophosphamide,doxorubicin,vincristine,prednisolone chemotherapy.However,he died within one month.CONCLUSION Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment.Repeated biopsy is the most important for early diagnosis and treatment.
基金supported in part by grants from the National High Technology Research and Development Program of China(863 Program)(No.2012AA02A502)a State Key Laboratory Grant at Sun Yat?sen University Cancer Center
文摘Introduction: The rearrangement of the anaplastic lymphoma kinase(ALK) gene accounts for approximately 1%–6% of lung adenocarcinoma cases and deines a molecular subgroup of tumors characterized by clinical sensitivity to ALK inhibitors such as crizotinib. This study aimed to identify the relationship between ALK rearrangement and the clinico?pathologic characteristics of non?small cell lung cancer(NSCLC) and to analyze the therapeutic responses of crizotinib and conventional chemotherapy to ALK rearrangement in NSCLC patients.Methods: A total of 487 lung cancer patients who underwent testing for ALK rearrangement in our department were included in this study. ALK rearrangement was examined by using fluorescence in situ hybridization(FISH) assay.Results: Among the 487 patients, 44(9.0%) were diagnosed with ALK rearrangement by using FISH assay. In 123 patients with adenocarcinoma who were non?smokers and of a young age(≤58 years old), the frequency of ALK rearrangement was 20.3%(25/123). Short overall survival(OS) was associated with non?adenocarcinoma tumor type(P = 0.006), poorly diferentiated tumors(P al growth factor rece= 0.001), advanced?stage tumors(P < 0.001), smoking history(P ptor(EGFR)(P = 0.008), and wild?type epidermrter time to cancer p= 0.008). Moreover, patients with poorly diferentiated and advanced?stage tumors had a shorogression compared with those with well diferentiated(P = 0.023) and early?stage tumors(P = 0.001), respectively.Conclusions: ALK?rearranged NSCLC tends to occur in younger individuals who are either non?smokers or light smokers with adenocarcinoma. Patients with ALK rearrangement might beneit from ALK inhibitor therapy.
基金Supported by The Italian Association for Cancer Research,AIRC 2013 IG-14249
文摘Aberrant forms of the anaplastic lymphoma kinase(ALK) are involved in the pathogenesis of several types of cancer, including anaplastic large cell lymphoma, non-small-cell lung cancer(NSCLC), inflammatory myofibroblastic tumors, colorectal cancer, neuroblastoma and others. In general, the ALK catalytic domain is rearranged and fused to a dimerization domain encoded by an unrelated gene. Less frequently, full-length ALK is activated by point mutations. The common theme is unregulated firing of ALK downstream signalling, leading to uncontrolled cell division and increased cell survival. ALK-driven tumors can be treated with Crizotinib, an orally available dual ALK/MET inhibitor, currently approved for advanced ALK-positive NSCLCs. Crizotinibtreated patients achieve high response rates, with an excellent toxicity profile. However, drug-resistant disease often develops, particularly in NSCLC patients. The processes leading to drug resistance include both ALKdependent(point mutations or gene amplification), as well as ALK-independent mechanisms, which are here briefly discussed. Recently, Ceritinib has been approved for Crizotinib-refractory NSCLC, further extending patients' survival, but resistance again emerged. Novel ALK kinase inhibitors are currently under clinical development, showing great promise for improved efficacy in drugresistance disease. It is opinion of the author that drugresistance is likely to arise under any treatment, due to intrinsic heterogeneity and adaptability of cancer. To prevent or delay this phenomenon, we need to treat less advanced disease, with drugs that are rapidly effective in order not to allow enough time for tumor evolution, and we want to have more and more drugs with nonoverlapping resistance profiles, for subsequent lines of targeted therapy. Finally, the use of drug combinations may exponentially decrease the chances of resistance.
文摘Kawasaki disease (KD) is an acute febrile systemic vasculitis occurring predominantly in young children less than 5 years of age. Although imperfectly known, the aetiopathogenesis of KD would be secondary to immunological abnormalities that could constitute a favourable terrain for neoplasms. We report on a case in a 2-year-old girl who presented clinical manifestations compatible with Kawasaki disease complicated by coronary aneurysm. Aetiopathological investigations revealed M. pneumoniae infection as specific IgM were present in the serum (Elisa). The patient was initially treated by intravenous immunoglobulins (IVIG) and aspirin to anti-inflammatory dose. Following a few days of desquamation, resolution of the symptomatology occurred. Four weeks later she developed disseminated tumorous syndrome. Lymph node biopsy revealed massive infiltration by large cells lymphomatous proliferation. Histologic and immunophenotypic findings were characteristic of ALK-1+ anaplastic large cell lymphoma. Four weeks later, the patient died from a severe nosocomial infection complicated by septic shock. Our observation is the first cases describing the association between anaplastic large cell lymphoma, KD and M. pneumoniae. Immunologic disorder due to KD and M. pneumoniae infection may play probably a central role for malignancy.
文摘BACKGROUND Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin’s lymphoma (NHL). ALCL is rare as a bone lesion and in pregnancy. CASE SUMMARY We present the first case of anaplastic lymphoma kinase (ALK)+ ALCL of the thoracic spine during pregnancy. A 25-year-old pregnant woman was presented to us at 24 wk’ gestation with severe back pain and weakness in the left lower limb. Imaging examination showed lesions at T10 and T11. She underwent posterior pedicle screw fixation and vertebroplasty. Pathological examination showed ALK+ ALCL. The patient chose to have therapeutic abortion after surgery and received chemotherapy in the hematology department. She now remains disease free with no neurological deficit after 30 mo’ follow-up. CONCLUSION ALK+ ALCL with the thoracic spine involvement is uncommon, especially in pregnancy. Many symptoms can be misunderstood during pregnancy;therefore, when a pregnant patient has persistent back pain or lower limb neurological symptoms, imaging examinations should be performed.
文摘Introduction: Anaplastic large-cell lymphoma occurring in the nasal cavity is a rare disease. The latest World Health Organization (WHO) Classification recognizes three distinct subtypes: primary systemic anaplastic lymphoma kinase positive been our case, primary systemic anaplastic lymphoma kinase negative and primary cutaneous types. Through this case study, we focus on the clinical presentation, treatment and prognostic characteristics of this pathology.Case Presentation: We report the case of a patient aged 32 years, who presented for seven months a runny nose associated with swelling of the face on the left side, without peripheral lymphadenopathy or general signs. A Blondeau scanner objectified a total filling of the frontal and left maxillary sinus, and a filling of the left nasal cavity. Complete resection of the tumor was performed. Histological examination was in favor of anaplastic large T-cell lymphoma anaplastic lymphoma kinase positive. The patient was stage IE according to Ann Arbor classification, with an International Prognostic Index estimated at one. Thus, the patient received six cycles of CHOP chemotherapy. Currently, he is in good loco-regional control with a decline of three months.Conclusion: The rarity of this case lies partly in the lymphomatous localization in the nasal cavity, and secondly in the anaplastic histology. It poses a diagnostic problem. So, we conclude that in case of any symptom of nasal cavities, it is necessary to explore and possibly biopsy if tumor, before surgery, because lymphomas are chemosensitive disease.