OBJECTIVE: To investigate the clinical characteristics, diagnosis, and treatment of one patient with primary adrenal natural killer/T-cell lymphoma (PANKTCL), and to strengthen the understanding of this rare type of lymphoma. METHODS: The clinical manifestations, diagnosis and treatment process, and prognosis of the patient admitted in our hospital were retrospectively analyzed. RESULTS: Combined with pathology, imaging, bone marrow examination， etc, the patient was diagnosed with PANKTCL (CA stage, stage II; PINK-E score 3, high-risk group). Six cycles of "P-GemOx+VP-16" regimen（gemcitabine 1 g/m² d1 + oxaliplatin 100 mg/m² d 1 + etoposide 60 mg/m² d 2-4 + polyethylene glycol conjugated asparaginase 3 750 IU d 5） was performed, and complete response was assessed in 4 cycles. Maintenance therapy with sintilimab was administered after the completion of chemotherapy. Eight months after the complete response, the patient experienced disease recurrence and underwent a total of four courses of chemotherapy, during which hemophagocytic syndrome occurred. The patient died of disease progression 1 month later. CONCLUSION PANKTCL is rare, relapses easily, and has a worse prognosis. The choice of the "P-GemOx+VP-16" regimen combined with sintilimab help to improve the survival prognosis of patient with non-upper aerodigestive tract natural killer /T-cell lymphoma.
Humans ; Treatment Outcome ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Retrospective Studies ; Etoposide ; Neoplasm Recurrence, Local/drug therapy* ; Asparaginase ; Deoxycytidine ; Lymphoma, T-Cell, Peripheral/drug therapy* ; Lymphoma, Extranodal NK-T-Cell/therapy* ; Oxaliplatin/therapeutic use*

Aminopyridines ; Antineoplastic Combined Chemotherapy Protocols ; Benzamides ; Humans ; Leukemia, Myeloid, Acute/drug therapy* ; Lymphoma, T-Cell, Peripheral/drug therapy*

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.
Aged* ; Comorbidity ; Cyclophosphamide ; Doxorubicin ; Drug Therapy* ; Febrile Neutropenia ; Follow-Up Studies ; Humans ; L-Lactate Dehydrogenase ; Lymphoma, T-Cell, Peripheral* ; Neutropenia ; Prednisolone ; Survival Rate ; Thrombocytopenia ; Treatment Outcome ; Vincristine

A 72-year-old male presented with respiratory discomfort. A simple chest X-ray and abdominal computed tomography showed pleural effusion and multiple lymph node enlargement. The pleural effusion was determined by thoracentesis to be chylothorax. An inguinal lymph node biopsy showed peripheral T-cell lymphoma. Following three cycles of cyclophospamide, hydroxyl doxorubicin, vincristine, prednisolone (CHOP) chemotherapy, a partial response was observed. Chylothorax is an extremely rare complication of T-cell lymphoma. We present a case of peripheral T-cell lymphoma presenting with chylothorax. We suggest that clinicians should consider chylothorax when examining patients with lymphoma who present with atypical pleural effusion.
Aged ; Biopsy ; Chylothorax* ; Doxorubicin ; Drug Therapy ; Humans ; Lymph Nodes ; Lymphoma ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral* ; Male ; Pleural Effusion ; Prednisolone ; Thoracentesis ; Thorax ; Vincristine

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.
Aged ; Aminopterin/adverse effects/*analogs & derivatives/therapeutic use ; Antineoplastic Agents/adverse effects/*therapeutic use ; Bortezomib/adverse effects/*therapeutic use ; Drug Administration Schedule ; Drug Therapy, Combination ; Humans ; Lymphoma, T-Cell, Peripheral/diagnostic imaging/*drug therapy/pathology ; Male ; Neoplasm Recurrence, Local ; Neutropenia/etiology ; Positron Emission Tomography Computed Tomography

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.
Adrenal Cortex Hormones ; Biopsy ; Diplopia ; Drug Therapy ; Female ; Herpesvirus 4, Human ; Humans ; Lymph Nodes ; Lymphocytes ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral* ; Middle Aged ; Mortality ; Orbit* ; Prognosis

BACKGROUND: This study aimed to assess the treatment outcomes of ifosphamide, mesna, etoposide, and prednisolone (IMEP) combination regimen as a front-line chemotherapy in patients with peripheral T-cell lymphomas (PTCLs). METHODS: Clinical data of 38 newly diagnosed PTCLs patients who underwent IMEP at Busan Paik Hospital from January 2002 to December 2013 were retrospectively analyzed. RESULTS: The overall response rate was 68.5%, with 21 (55.3%) complete response/complete response unconfirmed and 6 (15.8%) partial response (PR). The median follow-up duration was 25.5 months (range, 0.2-87.3). The median overall survival was not reached and 2-year survival rate was 67%. The median progression free survival was 23 months. The most frequently reported adverse effects higher than grade 3 were hematologic toxicities including neutropenia (68.4%), thrombocytopenia (42.1%). There was no treatment-related mortality. CONCLUSION: IMEP regimen is effective and safe as a front-line chemotherapy in patients with PTCLs.
Busan ; Disease-Free Survival ; Drug Therapy* ; Etoposide ; Follow-Up Studies ; Humans ; Lymphoma, T-Cell, Peripheral* ; Mesna ; Mortality ; Neutropenia ; Prednisolone ; Retrospective Studies ; Survival Rate ; Thrombocytopenia

OBJECTIVETo explore the clinical efficacy for treatment of patients with peripheral T cell lymphoma (PTCL) by HyperCVAD and CHOPCHOP-like protocols.

METHODSA total of 97 patients with peripheral T cell lymphoma were divided into observation group (50 cases) and control group (47 cases). The patients in observation group were treated by HyperCVAD, and the patients in the control group were treated by CHOP/CHOP-like protocol. The clinical efficacy, accumulate survival rate and side effect of the 2 groups were compared.

RESULTSThe remission rate in the observation group were higher than that in control group (P < 0.05); The PFS rate (1 year) in observation group was higher than that in control group (P < 0.05); the PFS rate (2 years, 3 years) was not significantly different (P > 0.05). The OS rate (1 year, 2 years, 3 years) did not show difference (P > 0.05); the number of patients with neutropenia in observation group was higher than that in control group (P < 0.05); the levels of CD4(+) CD45RA(+), CD4(+) CD45RO(+) in observation group were lower than those in control group (P < 0.05); the levels of CD4(+) and CD4(+)/CD8(+) in observation group was lower than those in control group (P < 0.05); the level of CD8(+) in observation group was higher than that in control group (P < 0.05); the incidence of pneumonia, cardiotoxicity, severe anemia, and thrombopenia were not significantly different (P > 0.05).

CONCLUSIONHypeCVAD protocol shows good clinical effects on the patients with peripheral T cell lymphoma, displaying a high remission rate and PFS rate (1 year), but it has a high incidence of neutropenia, thus it needs more attention in clinic.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Dexamethasone ; therapeutic use ; Doxorubicin ; therapeutic use ; Humans ; Lymphoma, T-Cell, Peripheral ; drug therapy ; Neutropenia ; complications ; Prednisone ; therapeutic use ; Remission Induction ; Survival Rate ; Treatment Outcome ; Vincristine ; therapeutic use

BACKGROUND: Among pediatric non-Hodgkin lymphomas, there are 4 major subtypes: Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma, and anaplastic large-cell lymphoma. Understanding of other rare subtypes derives only from small pediatric case series. We report our institutional experience with rare pediatric NHLs.METHODS: Thirty-six cases of rare NHL subtypes diagnosed at the Asan Medical Center from 1995 to 2015 were evaluated. We retrospectively reviewed the clinical and pathologic features and outcomes of these patients, excluding peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), and extranodal NK/T-cell lymphoma (ENKL), on which we have previously reported.RESULTS: There were 23 cases of T-cell lineage (13 PTCL, 6 ENKL, 2 subcutaneous panniculitis-like T-cell lymphoma, 1 primary cutaneous CD4+2016-11-22 small/medium sized T-cell lymphoma, 1 enteropathy-associated T-cell lymphoma) and 13 cases of B-cell lineage lymphoma (5 marginal zone lymphoma, 6 primary mediastinal large B-cell lymphoma, 2 immunoblastic and plasmablastic lymphoma). All patients were treated with chemotherapy with or without surgery, except 4 out of 5 patients with marginal zone lymphoma who received surgery only. Two patients died and 6 patients relapsed. One patient with primary mediastinal large B-cell lymphoma received autologous peripheral blood stem cell transplantation. The 5-year overall survival and event-free survival rates of rare pediatric NHL excluding PTCL, NOS, and ENKL was 80.0% and 72.0%, respectively.CONCLUSION: Children diagnosed with rare pediatric NHL other than PTCL, NOS, and ENKL showed variable incidence and treatment outcomes. Multicenter studies in larger cohorts are needed for better understanding of these rare NHL subtypes in childhood.
B-Lymphocytes ; Burkitt Lymphoma ; Child ; Chungcheongnam-do ; Cohort Studies ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Peripheral Blood Stem Cell Transplantation ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; T-Lymphocytes

No abstract available.
Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; B-Cell-Specific Activator Protein/metabolism ; Bone Marrow/metabolism/*pathology ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Endoscopy, Digestive System ; Female ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Genetic Loci ; Humans ; Liver/metabolism/pathology ; Lymphocytes/cytology/immunology ; Lymphoma, Large B-Cell, Diffuse/complications/*diagnosis/drug therapy ; Lymphoma, T-Cell, Peripheral/complications/*diagnosis/drug therapy ; Middle Aged ; Prednisone/therapeutic use ; Receptors, Antigen, T-Cell, gamma-delta/genetics ; Tomography, X-Ray Computed ; Vincristine/therapeutic use