Progress of gene alterations and molecular typing of peripheral T-cell lymphoma / 白血病·淋巴瘤

Peripheral T-cell lymphoma (PTCL) is a group of heterogeneous malignant tumors with poor prognosis, with a lack of standard treatment regimen and poor efficacy of traditional chemotherapy. Therefore, finding new and more effective therapeutic targets to improve the efficacy of PTCL is an urgent clinical problem. In recent years, as the exploration of PTCL at the genetic and molecular levels has intensified, novel therapeutic targets based on gene alterations and molecular typing have been identified. This article summarizes the research progress of main gene alterations and molecular typing of PTCL in recent years.

Early T-cell precursor acute lymphoblastic leukemia: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To improve the understanding of the diagnosis and treatment of early T-cell precursor acute lymphoblastic leukemia (ETP-ALL).Methods:The clinical data of a patient with ETP-ALL who was misdiagnosed as peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) admitted to the Second Hospital of Lanzhou University in October 2020 were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient who presented "inguinal lymphadenopathy" as the first symptom underwent lymph node biopsy and pathological examination at local hospital, and he was diagnosed as PTCL-NOS according to the consultation of another 2 hospitals. After 2 courses of chemotherapy (CHOPE regimen, GLD regimen, unknown specific medication and dosage), the therapeutic efficacy was poor. For further diagnosis and treatment, this patient came to Lanzhou University Second Hospital. Flow cytometry found blast cells in the bone marrow, and then other related examinations were completed, he was finally diagnosed as ETP-ALL. The chemotherapy regimens of Hyper-CVAD and EA were alternatively used, progressive disease (PD) occurred after 3 courses of treatment, and chidamide was added in the 4th and 5th courses of treatment, the disease still progressed, and the patient died after follow-up. The disease course of the patient was about 12 months.Conclusions:ETP-ALL has unique immunophenotypic characteristics. ETP-ALL patients have a low remission rate after conventional induction therapy, high recurrence rate and poor prognosis. Currently, there is no effective standard treatment regimen, and allogeneic hematopoietic stem cell transplantation or timely addition of new drugs may improve the prognosis.

Moxibustion Successfully Improved Chemotherapy-induced Peripheral Neuropathy in a Patient with Malignant Lymphoma / 日本東洋医学雑誌

A 70-year-old woman was diagnosed with peripheral T-cell lymphoma and received chemotherapy. She developed chemotherapy-induced peripheral neuropathy (CIPN), and her chief complaint was numbness of her fingertips and toes. However, the symptoms did not change even 9 months after the chemotherapy was completed. Our Kampo diagnosis was dual deficiency of qi (ki) and blood. Hence, we applied pedestal moxibustions to the following acupuncture points : SP 6 (Sanyinjiao), Ex-LE 10 (Bafeng), Ex-UE 9 (Baxie), CV 4 (Guanyuan). Her symptoms improved rapidly after moxibustion treatment. There were neither adverse events nor relapse of the numbness. Moxibustion treatment might reduce symptoms of CIPN.

Clinico-pathological analysis of lymphoproliferative disorders: a 3 year study

Background: The incidence of lymphoproliferative disorders has increased in many parts of the world. Newer subtypes have been identified by the new WHO classification. Accurate subtyping of lymphomas is crucial for prompt treatment. Objective of the study was to assess the clinicopathological pattern of lymphoproliferative diseases diagnosed in Rajagiri hospital over a period of 3 years.Methods: A retrospective study on all patients who were diagnosed with lymphoma in Rajagiri hospital during January 2016 to December 2018 was conducted and the data were reviewed and analyzed.Results: A total of 151 patients were included in the study. Majority of the subjects (63%) were males. The predominant age group affected was 61-80 years. Mean age group was 58.46 years (SD=19.05 years). Most common presenting symptom was painless lymphadenopathy. B symptoms were seen in 18% of subjects and was found to be more commonly associated with B cell Non-Hodgkin lymphoma. Most common lymph node involved was cervical lymph node, while the most common extra nodal site was bone marrow. Most common lymphoproliferative disorder was Diffuse large B cell Lymphoma. Lymph node involvement was found in 74% of patients, while hepatomegaly and splenomegaly were seen in 11% and 18.5% of patients. 20% of patients had secondary bone marrow involvement. 8 patients developed recurrent lesions involving other organs.Conclusions: Clinicopathologic patterns of lymphoproliferative disorders vary across various regions. A proper understanding of demographical distribution of lymphomas is very essential, as it can provide valuable clues for accurate diagnosis and treatment.

Primary Peripheral Gamma Delta T-Cell Lymphoma of the Central Nervous System: Report of a Case Involving the Intramedullary Spinal Cord and Presenting with Myelopathy

Primary central nervous system lymphoma of T-cell origin (T-PCNSL) is rare, and its clinicopathological features remain unclear. Peripheral T-cell lymphoma of γδ T-cell origin is an aggressive lymphoma mainly involving extranodal sites. Here, we report a case of γδ T-PCNSL involving the intramedullary spinal cord and presenting with paraplegia. A 75-year-old Korean woman visited the hospital complaining of back pain and lower extremity weakness. Magnetic resonance imaging revealed multifocal enhancing intramedullary nodular lesions in the thoracic and lumbar spinal cord. An enhancing nodular lesion was observed in the periventricular white matter of the lateral ventricle in the brain. There were no other abnormalities in systemic organs or skin. Laminectomy and tumor removal were performed. The tumor consisted of monomorphic, medium-to-large atypical lymphocytes with pale-to-eosinophilic cytoplasm. Immunohistochemically, the tumor cells were CD3(+), TCRβF1(-), TCRγ(+), CD30(-), CD4(-), CD8(-), CD56(+), TIA1(+), granzyme B(+), and CD103(+). Epstein-Barr virus in situ was negative. This case represents a unique T-PCNSL of γδ T-cell origin involving the spinal cord.

Cluster Containing More Than 20 CD3-Positive Cells in Bone Marrow Biopsy Is a Candidate Prognostic Indicator in Peripheral T-Cell Lymphoma, Not Otherwise Specified

Assessment of bone marrow (BM) involvement in peripheral T-cell lymphoma, not otherwise specified (PTCL) is straightforward in cases of extensive involvement but difficult in cases of minimal to partial involvement. We evaluated the usefulness of CD3 as an immunohistochemical marker for assessing BM involvement in PTCL patients. BM biopsies of 92 PTCL patients were immunohistochemically stained for CD3, CD4, CD8, CD20, and CD56, and evaluated by two hematopathologists. CD3 positivity was graded according to the proportion of CD3-positive cells and the number of CD3-positive cells in a cluster. These criteria were used to determine the cut-offs at which significant differences in progression-free survival (PFS) and overall survival (OS) were observed. Multivariate analysis controlling the International Prognostic Index (IPI) score and its individual factors revealed that >20 CD3-positive cells in a cluster adversely affected PFS (relative risk [RR], 2.1; 95% confidence interval [CI], 1.0–4.3; P=0.047) and OS (RR, 2.4; 95% CI, 1.1–5.1; P=0.028) independent of IPI score. A cluster with >20 CD3-positive cells is a candidate indicator for BM involvement in PTCL.

A Case of Peripheral T-cell Lymphoma, Unspecified Presenting as Multiple Ulcerative Tumors / 대한피부과학회지

Peripheral T cell lymphoma, unspecified (PTCL-U) comprises a heterogenous group of mature T-cell lymphomas that do not match with any defined T-cell entities in the current classification system. A 68-year-old man presented with extensive erythematous to brownish ulcerative tumors with yellowish discharge on the neck, trunk, and both upper extremities that had persisted for the past 7 months. Histological findings showed medium- to large-sized pleomorphic lymphocytes with cellular atypia infiltrating the deep dermis and subcutis. Immunohistochemical analysis of specimens from this patient revealed positive staining for CD2, CD45, and granzyme B and mildly positive staining for CD3, CD4, CD30, and CD79a. Based on these clinico-pathological findings, the patient was finally diagnosed with PTCL-U. We report herein a rare case of PTCL-U presenting as multiple ulcerative tumors.

Peripheral T-cell Lymphomas: Updates in Allogeneic Hematopoietic Stem Cell Transplantation / 中华医学杂志(英文版)

Objective@#Peripheral T-cell lymphomas (PTCLs) confer dismal prognosis and no consensus has been established on the role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) due to its rarity and heterogeneity. The purpose was to review key points of allo-HSCT for PTCLs, including indication, times of transplantation, conditioning regimen, graft versus host disease prophylaxis, and treatment of relapse.@*Data Sources@#A comprehensive search in PubMed and Cochrane up to February 28, 2018, with the keywords "Peripheral", "T", "Lymphoma", and "Transplantation" was done.@*Study Selection@#Relevant articles including HSCT for PTCLs were carefully reviewed.@*Results@#Promising data have been reported from advances in transplant technology and more and more PTCLs patients with poor prognosis could benefit from allo-HSCT.@*Conclusion@#Allo-HSCT is a useful choice for patients with refractory/relapsed PTCLs or high-risk new diagnosed PTCLs.

Current status of allogeneic hematopoietic stem cell transplantation for treatment of peripheral T-cell lymphoma / 白血病·淋巴瘤

Peripheral T-cell lymphoma (PTCL) is a kind of heterogeneous neoplasms mostly with strong invasiveness, a tendency of recurrence and drug resistance and poor prognosis. Five year disease-free-survival (DFS) rate of PTCL is less than 30% under the treatment setting of chemotherapy and autologous stem cell transplantation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has a role of a graft-versus-lymphoma effect in the treatment of PTCL, meanwhile, long DFS rate of allo-HSCT for the treatment of relapsed and refractory PTCL reaches 35%-50%. Therefore, allo-HSCT is an effective treatment method for PTCL patients.

Treatment outcomes of dose-attenuated CHOP chemotherapy in elderly patients with peripheral T cell lymphoma

BACKGROUND: While cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) is the most commonly used chemotherapeutic regimen for patients with peripheral T-cell lymphomas (PTCLs), elderly patients are more vulnerable to associated toxicities. We evaluated the efficacy and safety of dose-attenuated CHOP in elderly patients with PTCL. METHODS: Patients with PTCL aged >70 years or 65–70-years with comorbidities were treated with dose-attenuated CHOP (cyclophosphamide: 562.5 mg/m2, doxorubicin: 37.5 mg/m2, vincristine: 1.4 mg/m2, and prednisolone: 100 mg for five days; 25% reduced dose of cyclophosphamide and doxorubicin vs. full-dose CHOP) as first-line therapy were included. RESULTS: Forty-four patients (median age, 74 yr) were analyzed. The majority (N=42, 95.5%) had advanced stage disease and 36 (81.8%) were classified as high/high-intermediate risk by the international prognostic index. The overall response rate was 61.4%, and 21 patients achieved complete response (47.7%). With median follow-up period of 28.8 months, the estimated two-year progression-free and overall survival rates were 36.7% and 46.6%, respectively. Grade 3/4 neutropenia and thrombocytopenia occurred in 26.9% and 7.4% of 204 total cycles, which affected 76.7% and 25.6% of the patients, respectively. Nineteen patients (44.2%) experienced febrile neutropenia, and six died due to treatment-related toxicities. High lactate dehydrogenase levels and an involvement of >1 extranodal sites were prognostic indicators of poor survival. CONCLUSION: Dose-attenuated CHOP does not compromise treatment efficacy but retains significant toxicity. Our results suggest that some patients can be effectively treated with dose-attenuated CHOP, however a novel therapy for elderly patients with PTCL is required.

The correlation between platelet-lymphocyte ratio and the prognosis of patients with peripheral T-cell lymphoma, unspecified / 肿瘤

Objective: To identify the correlation between pre-treatment per ipheral blood platelet-l ymphoc y te rat io (PLR) and the clinicopathological features of the patients with peripheral T cell lymphoma, unspecified (PTCL-U), and to explore the prognostic significance of PLR in these patients. Methods: The clinical characteristics and survival state of 163 patients diagnosed of PTCL-U in Tianjin Medical University Cancer Institute and Hospital were analyzed retrospectively. All patients were divided into low PLR group (PLR < 170, n = 80) and high PLR group (PLR ≥ 170, n = 83) based on the receiver operating characteristic (ROC) curve's cut-off value. The relationship between pre-treatment PLR and the prognosis of these patients was analyzed. Results: In all 163 patients with PTCL-U, the 5-year overall sarvival (OS) and progression-free survival (PFS) rates of patients in low PLR group (76.3% and 60.0%, respectively) were higher than those in high PLR group (10.8% and 8.4%, respectively, both P < 0.05). Age, Eastern Cooperative Oncology Group performance status (ECOG PS), B symptoms, serum lactate dehydrogenase (LDH) level, platelet and PLR were remarkably relevant to OS (all P < 0.01). Age, serum LDH level, platelet and PLR were independent prognostic features for patients with PTCL-U (all P < 0.05). Conclusion: The peripheral blood PLR at pre-treatment is distinctly relevant to the prognosis of patients with PTCL-U. An elevated PLR predicts poor prognosis of these patients.

Expression of TET2 and DNMT3A in peripheral T cell lymphoma and their significance / 临床与实验病理学杂志

Purpose To investigate the expression of TET2 and DNMT3A in patients with peripheral T cell lymphoma (PTCL) and the relationship to immunophenotypes of PTCL.Methods Using a panel of immunohistochemical markers (CD3,CD4,CD10,BCL-6,CXCL-13,CD30,ALK),all cases of PTCLs were further divided into four groups,including angioimmunoblastic T cell lymphoma (AITL),peripheral T cell lymphoma,not otherwise specified (PTCL-NOS),anaplastic lymphoma kinase negative anaplastic large cell lymphoma (ALK-ALCL) and anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALK + ALCL).The expression of TET2 and DNMT3A in 89 cases of PTCL was detected by immunohistochemical analysis.Results 89 cases were divide into four subtypes,AITL (36/89),PTCL-NOS (26/89),ALKALCL (18/89),and ALK + ALCL (9/89).Immunohistochemistry staining revealed higher cytoplasmic expression of TET2 and DNMT3A in AITL than that of in PTCL-NOS and ALCL (P ＜ 0.05).And the nuclear expression of DNMT3A in patients with AITL was higher than that of PTCL-NOS and ALCL (P ＜ 0.05).The cytoplasmic expression of TET2 was positively related with both cytoplasmic and nuclear expression of DNMT3A in patients with AITL (P ＜ 0.05).Conclusion TET2 combined with DNMT3A could be used as markers in AITL diagnosis,which could provide new strategy for AITL diagnosis.

Adult T-cell leukemia/lymphoma / Leucemia/linfoma de células T do adulto

Summary Adult T-cell leukemia/lymphoma (ATL) is a malignancy of mature CD4+ T-cells caused by human T-cell lymphotropic virus type 1 (HTLV-1). Twenty million people are believed to be infected throughout the world, mostly in Japan, Africa, the Caribbean, and South America, particularly in Brazil and Peru. ATL affects about 5% of infected individuals and is classified in the following clinical forms: acute, lymphoma, primary cutaneous tumoral, chronic (favorable and unfavorable), and smoldering (leukemic and non-leukemic). Although it is considered an aggressive disease, there are cases with a long progression. We emphasize the importance of clinical classification as an indispensable element for evaluating prognosis and appropriate therapeutic approach. Since several cases have been published in Brazil and this disease is still poorly known, we decided to make a review paper for dissemination of clinical, hematological and pathological aspects, diagnosis, and therapy. The best way to reduce the occurrence of ATL would be halting the transmission of the virus through breastfeeding.

Resumo A leucemia/linfoma de células T do adulto (LLcTA) é uma neoplasia de células T maduras CD4+ causada pelo vírus linfotrópico para células T humanas tipo 1 (HTLV-1). Acredita-se que existem cerca de 20 milhões de pessoas infectadas em todo o mundo, principalmente no Japão, na África, no Caribe e na América do Sul, particularmen te no Brasil e no Peru. A LLcTA acomete cerca de 5% dos indivíduos infectados e classifica-se nas seguintes formas clínicas: aguda, linfomatosa, tumoral primária de pele, crônica (favorável e desfavorável) e indolente (leucêmica e não leucêmica). Embora seja considerada uma doença agressiva, há casos com longa evolução. Salientamos a importância da classificação clínica como elemento im prescindível para avaliação do prognóstico e conduta terapêutica adequada. Como já foram publicados vários casos no Brasil e essa doença ainda é pouco conhecida, decidimos fazer um trabalho de revisão para divulgar os seus aspectos clínicos, hematológicos, anatomopatológi cos, diagnósticos e terapêuticos. O melhor meio de redu zir a ocorrência de LLcTA seria sustando a transmissão vertical do vírus pela amamentação.

A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features

PURPOSE: To report a case of complete remission of primary orbital peripheral T-cell lymphoma with panniculitis-like features after chemotherapy. CASE SUMMARY: A 57-year-old healthy female presented with periorbital swelling and symptoms of diplopia. The patient was first treated with high-dose systemic corticosteroids, however, symptoms persisted. Therefore, anterior orbitotomy with excisional biopsy was performed for diagnostic purposes. On microscopic examination, the excised mass showed localized dense lymphocyte infiltrates, and cytologic atypia was observed under a high-power field. On immunehistochemical examination, tumor cells were positive for CD3 and CD8 but negative for CD4, CD20 and CD56. Based on histopathological results, primary orbital peripheral T-cell lymphoma with panniculitis-like features was diagnosed. Additionally, molecular pathological testing was positive for Epstein-Barr virus. Subsequently, the patients underwent chemotherapy and complete remission was obtained. CONCLUSIONS: Peripheral T-cell lymphoma often manifests as systemic symptoms, including lymph node enlargement and B symptom. The primary form of the disease in an orbit is very rare, and has a poor prognosis with a high mortality rate because the disease quickly progresses. Herein, the authors report a rare case of a healthy patient without any past medical history who achieved complete remission of a fast-growing primary orbital T-cell lymphoma with no preceding systemic symptoms.

Pralatrexate in Combination with Bortezomib for Relapsed or Refractory Peripheral T Cell Lymphoma in 5 Elderly Patients

Peripheral T cell lymphoma (PTCL) is a heterogeneous group of aggressive lymphomas with poor prognosis. Elderly (age ≥ 65years) patients generally have impaired bone marrow function, altered drug metabolism, comorbidities, and poor functional status. Thus, treatment of elderly patients with relapsed or refractory PTCL remains a challenge for clinicians. A recent study disclosed that pralatrexate has a synergistic effect in combination with bortezomib. Weekly pralatrexate and bortezomib were administered intravenously for 3 weeks in a 4-week cycle. Of 5 patients, one achieved complete response after 4 cycles which has lasted 12 months until now. Another patient attained partial response after 2 cycles. Only 1 patient experienced grade 3 thrombocytopenia and neutropenia. Two patients suffered from grade 3 mucositis. Combination therapy with pralatrexate and bortezomib may be used as a salvage therapy for relapsed or refractory PTCL in the elderly with a favorable safety profile.

Clinical analysis of Hyper-CVAD/MA regimen and CHOP/CHOP-like regimen in the treatment of 78 patients with peripheral T-cell lymphoma, not otherwise specified (PT-CL-NOS) / 中国肿瘤临床

Objective:To compare the therapeutic efficacy and safety of Hyper-CVAD/MA regimen and CHOP/CHOP-like regimen in the treatment of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Methods:The 78 primary PTCL-NOS patients who were initially diagnosed and treated in Tianjin Medical University Cancer Institute and Hospital and Tianjin Union Medical Center from June 2004 to June 2012 were retrospectively analyzed. The patients were then divided into two groups:Hyper-CVAD/MA group (n=21) and CHOP/CHOP-like group (n=57). Curative efficacies and toxicities were analyzed by Chi-square test, and survival was estimated by Ka-plan-Meier method. Results: In the Hyper-CVAD/MA group, complete response (CR) was 42.9%, overall response rate (ORR) was 85.7%, median progression-free survival (PFS) was 20 months, and the three-year overall survival (OS) was 56.9%. In the CHOP/CHOP-like group, the CR, ORR, and three-year OS were 28.1%, 59.6%, and 49.6%, respectively, and the median PFS was 13 months. Compara-tive analysis showed that the ORR and three-year OS were statistically significant (P0.05). The incidence rates ofⅢ/Ⅳneutrocytopenia and thrombocytopenia in Hyper-CVAD/MA group (66.7%and 61.9%, respectively) were significantly higher than those of the CHOP/CHOP-like group (22.8%and 14.0%, respec-tively) (P<0.05). Conclusion:Hyper-CVAD/MA regimen can achieve satisfactory efficacy in parents with PTCL-NOS, and toxicity can be controlled with granulocyte colony stimulating factor (G-CSF).

The expressions and clinical significance of PD-L1 and PD-1 in peripheral T-cell lymphoma / 天津医药

Objective To investigate the expression levels and clinical significance of programmed cell death 1 ligand (PD-L1) and programmed cell death factor-1 (PD-1) in peripheral T-cell lymphoma (PTCL). Methods Immunohistochem?istry was used to detect expression levels of PD-L1 and PD-1 in PTCL (test group, n=51) and benign proliferative lesions of lymph node tissues (control group, n=20). The correlations of PD-L1 and PD-1 expressions with clinical pathological param?eters and prognosis were analyzed between two groups. Results The expression level of PD-L1 was significantly higher in PTCL group than that in control group (74.51%vs 35.00%,χ2=9.662, P<0.05). The positive expression of PD-1 was signifi?cantly higher in PTCL group than that in control group (66.67%vs 25.00%,χ2=10.074, P<0.05). There were significant dif?ferences in PD-L1 and PD-1 expressions between different peripheral lactate dehydrogenase (LDH) levels of PTCL group (P<0.05). After two cycles of CHOP or ECHOP treatments, the response rate (RR) was higher in PD-L1 negative group than that in positive group (84.6%vs 47.4%,χ2=5.478, P<0.05), and RR was higher in PD-1 negative group than that in positive group(82.4%vs 44.1%,χ2=6.755, P<0.05). The median overall survival (OS) time was higher in PD-L1 negative group than that in positive group (29.8 months vs 17.6 months,χ2=4.413, P<0.05) and the median OS time was higher in PD-1 negative group than that in positive group (29.8 months vs 17.6 months,χ2=8.293, P<0.05). Conclusion There are high expression levels of PD-L1 and PD-1 in peripheral T-cell lymphoma, which is closely related with the elevated LDH in peripheral blood, poor response rate and shorter OS. Therefore, the expression levels of PD-L1 and PD-1 can be used as factors of worse effect of chemotherapy and poor prognosis.

Advances in therapeutic options of peripheral T-cell lymphoma / 中国肿瘤临床

To date, the treatment of peripheral T-cell lymphomas (PTCL) has lagged behind B-cell malignancies. Traditionally, para-digms for diffuse large B-cell lymphoma were applied to patients with PTCL, but the outcomes were poor. Recently, the FDA has ap-proved four drugs for patients with relapsed/refractory PTCL, and the Japanese government has approved of anti-CCR4 monoclonal an-tibody for patients with adult T-cell leukemia/lymphoma. Clinical studies are exploring the combination of these new agents into stan-dard CHOP-based regimens for patients with newly diagnosed PTCL. Recent studies have revealed that PTCL may be associated with epigenetic dysregulation and is thus sensitive to histone deacetylase inhibitors. These advances provide a new understanding of PTCL, whose therapeutic options will be presented in this review.

Non-mycosis Peripheral T-Cell non-Hodgkin Lymphoma involving the Skin.

A 64 years old male presented with reddish lesions all over the body of 1 month duration, high grade fever with evening rise of temperature and chills. No lymphadenopathy or hepatosplenomegaly were noted. Multiple infiltrated erythematous and hyperpigmented patches and plaques were present on the face, trunk and extremities along with few oral erosions. Histopathology from skin showed features of mycosis fungoides (MF). A further workup with Immunohistochemistry was suggestive of peri pheral T-cell lymphoma, not otherwise specified diag nosis (PTCLNOS). We report a case of PTCLNOS in a man mimicking MF clinically and histopathologically.

Pancreatic Lymphoma Masquerading as Autoimmune Pancreatitis / 대한췌담도학회지

Accurate diagnosis of autoimmune pancreatitis (AIP) is important to clinicians since it is difficult to differentiate AIP from pancreatic malignancies. Furthermore, unlike pancreatic malignancies, AIP has dramatic response to steroids. A 61-years-old man presented with acute pancreatitis. Imaging studies showed two separate pancreatic masses, irregular narrowing of main pancreatic duct, and a renal mass that highly suggested AIP. Endoscopic ultrasound-guided core needle biopsy of the pancreatic masses and ultrasound-guided biopsy of the renal mass revealed peripheral T-cell lymphoma. The patient is currently undergoing chemotherapy. We present a case of pancreatic lymphoma masquerading as AIP with literature review.