Absence of enhancement in a lesion does not preclude primary central nervous system T-cell lymphoma:A case report

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.

Review of lymphoma in the duodenum: An update of diagnosis and management

The presentation,subtype,and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract.We searched PubMed for publications between January 1,2012 and October 10,2022,and retrieved 130 articles relating to duodenal lymphoma.A further 22 articles were added based on the manual screening of relevant articles,yielding 152 articles for full-text review.The most predominant primary duodenal lymphoma was follicular lymphoma.In this review,we provide an update of the diagnosis and management of representative lymphoma subtypes occurring in the duodenum:Follicular lymphoma,diffuse large B-cell lymphoma,extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue,mantle cell lymphoma,and Tcell lymphomas.

Plasmacytosis mimicking multiple myeloma in angioimmunoblastic T-cell lymphoma:A case report and review of literature

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.

Monomorphic epitheliotropic intestinal T-cell lymphoma with bone marrow involved: A case report

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.

Primary anaplastic lymphoma kinase-positive large B-cell lymphoma of the left bulbar conjunctiva: A case report

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.

Rare primary colonic T cell lymphoma with curative resection by endoscopic submucosal dissection:A case report

BACKGROUND The gastrointestinal tract is a well-known extranodal site of lymphoma.B-cell lymph-oma is the most common type,while T-cell lymphoma is uncommon.Primary gastrointestinal lymphoma mainly occurs in the stomach and small intestine,and the colon is less frequently involved,especially in females.CASE SUMMARY A 45-year-old woman was admitted to our hospital for physical examination.Gastroenteroscopy revealed a visible pedunculated polyp in the transverse colon,for which endoscopic submucosal dissection(ESD)was performed.Pathology suggested highly active proliferation of T lymphocytes with atypical hyperplasia.CONCLUSION A middle-aged female patient was found to have colonic T-cell lymphoma by endoscopy.The lesion was successfully removed by ESD,and the surgical margin was negative.It is essential to raise awareness of colonic T-cell lymphoma and choose the appropriate treatment.

Non-Hodgkin's lymphoma involving chronic difficult-to-heal wounds:A case report

BACKGROUND Non-Hodgkin's lymphoma(NHL)is a malignant tumor that originates from the lymphoid tissues and can potentially affect numerous organs within the body.Among these,the skin stands out as one of the primary sites affected by NHL,often presenting with multiple extra-nodal manifestations.In this report,we present an unusual case of NHL involving chronic wounds in the lower extremities that were difficult to heal.The scars were successfully treated using radiotherapy in combination with extended excision debridement and peroneal artery perforator flap grafting,resulting in satisfactory outcomes.CASE SUMMARY A 19-year-old male patient presented with ulceration of the skin on the left calf near the ankle accompanied by purulent discharge.Subsequent pathologic biopsy confirmed a diagnosis of NHL(extranodal NK/T-cell lymphoma,nasal type).Initial treatment comprised local radiotherapy and wound care;however,the wound exhibited prolonged non-healing.Consequently,the patient underwent a series of interventions including radiotherapy,wound enlargement excision debridement,and peroneal artery perforator flap grafting.Ultimately,successful healing was achieved with favorable postoperative outcomes characterized by good texture of the flap without any signs of rupture or infection.CONCLUSION The combination of radiotherapy,wound enlargement excision debridement,and peroneal artery perforator flap grafting may present a favorable treatment modality for chronic non-healing lower leg wounds resulting from NHL.

Primary cutaneous B cell lymphoma: Clinical features, diagnosis and treatment

Primary cutaneous B cell lymphoma(PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of primary cutaneous lymphomas accounting for 25%-30%. Since the prognosis and treatment differ from systemic lymphomas involving the skin, differential diagnosis is very important. PCBCL is a heterogeneous group of disease comprising different B cell lymphomas with distinct treatment and prognosis. PCBCL is divided into 5 subclasses according to World Health Organization and European Organization of Research and Treatment of Cancer classification. Primary cutaneous marginal zone lymphoma and primary cutaneous follicle centerlymphoma are indolent forms and often confined to skin at presentation and during the course of the disease. But primary cutaneous diffuse large B cell lymphoma, leg type and intravascular large B cell lymphoma are more aggressive forms that may disseminate to extra-cutaneous tissues. There is not a treatment consensus since they are rare entities. Local therapies like radiotherapy, surgery or intralesional steroids are options for localized disease in indolent forms. More disseminated disease may be treated with a systemic therapy like single agent rituximab. However combination chemotherapies which are used in systemic lymphomas are also required for aggressive PCBCL. Although indolent forms have relatively better prognosis, early relapses and disseminated diseases are mostly observed in aggressive form with a consequent poor prognosis.

2022 Chinese expert consensus and guidelines on clinical management of toxicity in anti-CD19 chimeric antigen receptor T-cell therapy for B-cell non-Hodgkin lymphoma

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.

Diagnosis and surgical treatment of primary hepatic lymphoma

AIM: To assess the benefi ts and limits of surgery for primary hepatic lymphoma (PHL), and probability of survival after postoperative chemotherapy. METHODS: A retrospective analysis was undertaken to determine the results of surgical treatment of PHL over the past 8 years. Only nine patients underwent such treatment. The detailed data of diagnosis, treatment, and prognosis were carefully studied. RESULTS: All patients were mistaken as having α-fetoprotein-negative hepatic cancer before pathological diagnosis. The mean delay time between initial symptoms and final diagnosis was 26.8 d (range:14-47 d). Hepatitis B virus infection was noted in 33.3% of these patients. Most of the lesions were found to be restricted to a solitary hepatic mass. The surgical procedure performed was left hepatectomy in five cases, including left lateral segmentectomy in three. Right hepatectomy was performed in three cases and combined procedures in one. One patient died on the eighth day after surgery,secondary to hepatic insufficiency. The cumulative 6-mo, 1-year, and 2-year survival rates after hepatic surgery were, respectively, 85.7%,71.4%, and 47.6%. One patient survived for>5 years after surgery without any signs of recurrence until latest follow-up, who received routine postoperative chemotherapy every month for 2 years and then regular follow-up. By univariate analysis, postoperative chemotherapy was a significant prognostic factor that influenced survival (P=0.006). CONCLUSION: PHL is a rare entity that is often misdiagnosed, and has a potential association with chronic hepatitis B infection. The prognosis is variable, with good response to early surgery combined with postoperative chemotherapy in strictly selected patients.

Chinese Society of Clinical Oncology(CSCO) diagnosis and treatment guidelines for malignant lymphoma 2021(English version)

1. General guidelines2. Diagnosis3. Staging4. Treatment4.1 Diffuse large B-cell lymphoma(DLBCL)4.2 Follicular lymphoma(FL)4.3 Mantle cell lymphoma(MCL)4.4 Marginal zone lymphoma(MZL)4.5 Burkitt lymphoma(BL)4.6 CLL/Small lymphocytic lymphoma(SLL)4.7 Extra-nodal natural killer/T-cell lymphoma(ENKTCL), nasal type4.8 Peripheral T-cell lymphoma(PTCL)4.9 HL4.10 Primary central nervous system lymphoma5. Prognosis Lymphomas are a group of heterogeneous diseases.

CT diagnosis of 52 patients with lymphoma in abdominal lymph nodes

AIM： To assess CT manifestations and its diagnostic value for lymphoma in the abdominal lymph nodes （LALN）.METHODS： CT findings in 52 cases of LALN proved by surgery or biopsy, including Hodgkin＇s disease （HD） in 16 cases and non-Hodgkin＇s lymphoma （NHL） in 36 cases, were retrospectively analyzed.RESULTS： （1） CT manifestations based on distribution of the lesions of LALN： Solitary mass type was found in 10 cases, including solitary, round, uniform-density, enlarged lymph nodes in 3 cases; and multiple, enlarged lymph nodes fusing into singular Iobular mass in 7 cases. Thirty-feur cases of multiple-nodular type showed multiple, round, enlarged lymph nodes with uniform density and clear margins. Vessels-embedded signs, including mesenteric vessels, renal vessels, abdominal aorta or inferior vena cava, were seen in 6 cases, and duodenum-embedded signs were seen in 2 cases. Eight cases of diffuse type showed characteristic ＂cobblestone signs＂. （2） CT manifestations correlated with pathological type： CT manifestations of 12 cases of HD were different from those of 40 cases of NHL in distribution, size, quantity and fused lesion of enlarged lymph nodes. （3） Twenty-eight cases of 52 patients were accompanied with extra-nodal lymphoma in the abdomen, especially gastrointestinal lymphoma, which had characteristic CT findings. （4） In follow-up examinations, CT images showed uniform, heterogeneous or rim enhancement in 15 cases, and occasional calcifications accompanied with reduction of the lesion size and quantity in 12 cases, whereas the lesions disappeared in 3 cases after treatment.CONCLUSION： CT images show many characteristic manifestations valuable for qualitative diagnosis of LALN, and it is also helpful for pathological classification of LALN and therapeutic evaluation in follow-up of patients.

National guidelines for diagnosis and treatment of malignant lymphoma 2022 in China(English version)

Contents 1.Overview 2.Diagnosis of lymphoma 2.1 Clinical manifestations 2.2 Physical examination 2.3 Laboratory examination 2.4 Imaging examination 2.4.1 CT 2.4.2 MRI 2.4.3 PET-CT 2.4.4 Ultrasound 2.4.5 Isotope bone scan 2.4.6 Endoscopy 2.5 Pathological examination 2.5.1 Morphology 2.5.2 IHC 2.5.3 Fluorescence in situ hybridization(FISH)2.5.4 Antigen receptor gene rearrangement of lymphocytes 2.5.5 Others。

Potential model for differential diagnosis between Crohn's disease and primary intestinal lymphoma

AIM To evaluate the usefulness of different parameters to differentiate Crohn's disease(CD) from primary intestinal lymphoma(PIL).METHODS The medical records of 85 patients with CD and 56 patients with PIL were reviewed retrospectively. Demographic, clinical, laboratory, endoscopic, and computed tomographic enterography(CTE) parameters were collected. The univariate value of each parameter was analyzed. A differentiation model was established by pooling all the valuable parameters. Diagnostic efficacy was analyzed, and a receiver operating characteristic(ROC) curve was plotted.RESULTS The demographic and clinical parameters that showed significant values for differentiating CD from PIL included age of onset, symptom duration, presence of diarrhea, abdominal mass, and perianal lesions(P < 0.05). Elevated lactate dehydrogenase and serum β2-microglobulin levels suggested a PIL diagnosis(P < 0.05). The endoscopic parameters that showed significant values for differentiating CD from PIL included multiple-site lesions, longitudinal ulcer, irregular ulcer,and intraluminal proliferative mass(P < 0.05). The CTE parameters that were useful in the identification of the two conditions included involvement of ≤ 3 segments, circular thickening of the bowel wall, wall thickness > 8 mm, aneurysmal dilation, stricture with proximal dilation, "comb sign", mass showing the "sandwich sign", and intussusceptions(P < 0.05). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of the differentiation model were 91.8%, 96.4%, 93.6%, 97.5%, and 88.5%, respectively. The cutoff value was 0.5. The area under the ROC curve was 0.989.CONCLUSION The differentiation model that integrated the various parameters together may yield a high diagnostic efficacy in the differential diagnosis between CD and PIL.

Chinese guidelines for diagnosis and treatment of malignant lymphoma 2018(English version)

1. Overview2. Diagnosis of lymphoma2.1 Clinical manifestations2.2 Physical examination2.3 Laboratory examination2.4 Imaging examination2.4.1 CT2.4.2 MRI2.4.3 PET-CT2.4.4 Ultrasound2.4.5 Isotope bone scan2.5 Other specific examinations2.6 Pathological examinations2.6.1 Morphology2.6.2 IHC2.6.3 Fluorescence in situ hybridization (FISH)2.6.4 Antigen receptor gene rearrangement of lymphocytes2.6.5 Others.

Primary lymphoma of the liver-A complex diagnosis

A 59-year-old woman presented with the clinical symptoms and radiologic investigations of a liver lesion suspect of metastasis. However, postoperative histopathology revealed a primary hepatic lymphoma (PHL). The case of a patient with a solitary PHL, which was treated by resection and subsequent chemotherapy, will be discussed with a short overview of the literature.

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion:A case report

BACKGROUND Intestinal natural killer/T-cell lymphoma(NKTCL)is a rare and aggressive non-Hodgkin’s lymphoma,and its occurrence is closely related to Epstein-Barr virus infection.In addition,the clinical symptoms of NKTCL are not obvious,and the specific pathogenesis is still uncertain.While NKTCL may occur in any segment of the intestinal tract,its distinct location in the periampullary region,which leads clinicians to consider mimics of a pancreatic head mass,should also be addressed.Therefore,there remain huge challenges in the diagnosis and treatment of intestinal NKTCL.CASE SUMMARY In this case,we introduce a male who presented to the clinic with edema of both lower limbs,accompanied by diarrhea,and abdominal pain.Endoscopic ultrasound(EUS)showed well-defined homogeneous hypoechoic lesions with abundant blood flow signals and compression signs in the head of the pancreas.Under the guidance of EUS-fine needle biopsy(FNB)with 19 gauge or 22 gauge needles,combined with multicolor flow cytometry immunophenotyping(MFCI)helped us diagnose NKTCL.During treatments,the patient was prescribed the steroid(dexamethasone),methotrexate,ifosfamide,L-asparaginase,and etoposide chemotherapy regimen.Unfortunately,he died of leukopenia and severe septic shock in a local hospital.CONCLUSION Clinicians should enhance their understanding of NKTCL.Some key factors,including EUS characteristics,the right choice of FNB needle,and combination with MFCI,are crucial for improving the diagnostic rate and reducing the misdiagnosis rate.

Primary Lymphoma of Bone: Imaging Findings to Improve Diagnosis of a Rarely Considered Disease Prior to Biopsy

Objective: Primary lymphoma of bone (PLB) is a rare malignant bone tumor often presenting in the fifth-sixth decades involving appendicular long bones. Published radiological findings indicate that PLB typically presents as a moth-eaten osteolytic lesion with periosteal reaction, while MRI commonly demonstrates marrow infiltration with extraosseous extension. Given rarity and variable appearances, PLB may not be primarily considered prior to biopsy. Our objective was to evaluate preoperative imaging findings in effort to increase awareness and improve a perceived deficiency in preoperative diagnosis. Materials and Methods: Following IRB approval, retrospective review identified 60 patients with newly diagnosed bone lesions proven to represent PLB in accordance with WHO definition. Preoperative radiographs (n = 46), MRI (n = 33) and PET (n = 37) were independently reviewed by two radiologists. At radiography, lesions were classified: purely lytic, mixed, purely sclerotic, or occult;lytic lesions were graded utilizing Lodwick’s classification. At MRI, lesions were defined as focal or infiltrative and the presence or absence of extraosseous disease was recorded. Extraosseous masses were defined as small (1 cm) and subjectively correlated with degree of cortical destruction. At PET, lesions were recorded as FDG-avid or not. Primary radiograph reports when available (n = 33) were reviewed and exact wording of differential considerations was recorded. Results: Radiographs demonstrated mixed (n = 22), lytic (n = 15), and sclerotic (n = 8) appearances;one radiographically occult lesion was seen by MRI. Lytic lesions were graded: IB (n = 3), IC (n = 5), II (n = 4), and III (n = 3);none were IA. At MRI, 30 lesions were infiltrative and 3 were focal;11 were not associated with extraosseous extension, while 22 showed bony disease with small (n = 7) or large (n = 15) soft tissue mass. Of large masses, 13 demonstrated minimal cortical destruction. At PET, 36 demonstrated FDG uptake;one study was technically limited. Review of reports found that only 5 included “lymphoma” as a diagnostic consideration. Conclusion: Contrary to most published data, we suggest that PLB typically demonstrates some degree of osteosclerosis, often a mixed pattern of sclerosis and lucency;purely lytic lesions may be less common. Similar to existing reports, MRI commonly demonstrates marrow infiltration with extraosseous extension of disease, typically a large soft tissue mass with disproportionate (minimal) cortical destruction. Familiarity with these findings should improve preoperative consideration of PLB in the appropriate clinical scenario when a new osteoblastic lesion is identified.

Transcriptome sequencing analysis of ursolic acid-mediated proliferation suppression on cutaneous T-cell lymphoma cells

Background:Ursolic acid is a triterpenoid compound found in natural plants that exhibits antiproliferative effects in various cancer cells.Our study is the first to demonstrate the strong inhibitory effects of ursolic acid on the proliferation of cutaneous T-cell lymphoma(CTCL)cells.We aimed to further investigate the underlying mechanism of the proliferation inhibition induced by ursolic acid in CTCL cells using transcriptome sequencing.Methods:Cell counting kit-8 assays were used to observe the effects of six traditional medicine monomers on the proliferation of CTCL cells.Transcriptome sequencing was used to identify differentially expressed genes after ursolic acid treatment.Bioinformatics analysis was performed to determine the potential mechanism.Real-time quantitative PCR and western blotting analyses were performed to confirm the sequencing results and verify the possible mechanisms of ursolic acid-mediated proliferation inhibition in CTCL cells.Results:Ursolic acid exhibited the strongest inhibitory effect on the proliferation of CTCL cells among the six traditional medicine monomers.Transcriptome sequencing analysis showed that 2,466 genes were significantly altered.Combined with Kyoto Encyclopedia of Genes and Genomes functional enrichment analysis and protein-protein interaction network analysis,the interaction of various pathways and signaling molecules,such as tumor necrosis factor-α,NLR family pyrin domain containing 1,c-Jun N-terminal kinase,and melanoma differentiation-associated gene 5,accounted for the anti-tumor effects of ursolic acid in CTCL cells.Conclusion:Ursolic acid significantly inhibited the proliferation of CTCL cells,and our study laid a theoretical foundation for the future treatment of CTCL using ursolic acid.

Angioimmunoblastic T-cell lymphoma induced hemophagocytic lymphohistiocytosis and disseminated intravascular coagulopathy: A case report

BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here,we report a case of AITL induced hemophagocytic lymphohistiocytosis(HLH)and disseminated intravascular coagulopathy(DIC).CASE SUMMARY An 83-year-old man presented with fever and purpura of both lower limbs for one month. Groin lymph node puncture and flow cytometry indicated a diagnosis of AITL. Bone marrow examination and other laboratory related indexes indicated DIC and HLH. The patient rapidly succumbed to gastrointestinal bleeding and septic shock.CONCLUSION This is the first reported case of AITL induced HLH and DIC. AITL is more aggressive in older adults. In addition to male gender, mediastinal lymphadenopathy, anaemia, and sustained high level of neutrophil-to-lymphocyte ratio may indicate a greater risk of death. Early diagnosis, early detection of severe complications, and prompt and effective treatment are vital.