NK/T cell lymphoma, a rare type of non-Hodgkin's lymphoma, is a highly aggressive disease with poor prognosis. Clinically, it is classified into nasal, non-nasal, and aggressive lymphoma/leukemia subtypes. They are characterized by geographic distribution and are universally associated with Epstein-Barr virus (EBV) infection. Due to its low occurrence and dismal clinical outcome, no therapeutic strategy is currently identified in this disease. Combined chemotherapy and radiotherapy have better effects for stage I/II nasal NK cell lymphoma. As for stage III/IV nasal NK cell lymphoma and non-nasal, and aggressive subtypes, chemotherapy is the main treatment method. Recently, some studies have demonstrated promising outcomes in the selected cases by high-dose chemotherapy supplemented with auto- or allo-HSCT.
Humans ; Lymphoma, Non-Hodgkin ; classification ; therapy ; Lymphoma, T-Cell ; therapy

Humans ; Lymphoma, T-Cell, Peripheral ; diagnosis ; therapy

Angiocentric T cell lymphoma is a variant of peripheral T cell lymphoma characterized by an angiocentric and angioinvasive infiltrates, invasion of vascular walls, and occulsion of lumen by atypical lymphoid cells with varying degrees of cytologic atypia. We recently experienced a woman with angiocentric T cell lymphorma, who presented with multiple erythematous nodules of skin, subcutaneous mass on right face and peripheral polyneuropathy. At the skin biopy specimen, we could find angiocentric and angioinvasive infiltrate by atypical lymphoid cells which showed a positive immunohistochemical reaction for CD45RO that is one of the T cell markers and a negative reaction for B cell marker. She was treated with two cycles of combination chemotherapy composed of CVP (cyclophosphamide, vincristine, prednisolone), that resulted in complete remission.
Drug Therapy, Combination ; Female ; Humans ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Polyneuropathies* ; Skin ; Vincristine

Subcutaneous panniculitis-like T cell lymphoma (SPTL) is a distinctive cutaneous lymphoma characterized by an infiltration of subcutaneous tissue by neoplastic T cells, similar to panniculitis. It is well-established that patients who are diagnosed with SPTL usually respond poorly to chemotherapy, showing fatal outcome. As a first line treatment for SPTL, anthracycline-based chemotherapy was most frequently used. For the treatment of SPTL, the efficacy of cyclosporine A has been recently reported in relapsed SPTL after anthracycline-based chemotherapy. However, it is still not clear whether cyclosporine A can be used as a first-line treatment against SPTL. Here, we report a case of SPTL, which achieved complete remission for nine years after first-line cyclosporine A therapy. This study suggests that cyclosporine A can induce a complete long-term remission as a first-line treatment.
Cyclosporine* ; Drug Therapy ; Fatal Outcome ; Humans ; Lymphoma ; Lymphoma, T-Cell* ; Panniculitis ; Subcutaneous Tissue ; T-Lymphocytes

Cutaneous gamma/delta T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment, such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous gamma/delta T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis.
Drug Therapy, Combination ; Female ; Humans ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous ; Neoplasm Metastasis ; Recurrence ; T-Lymphocytes

Angiocentric T-cell lymphoma is a subtype of peripheral T-cell lymphoma and histologically characterized by angiocentric and angioinvasive infiltration of lymphoid cells with varying degrees of cytological atypia, leading to angiodestruction. This disease represents the malignant end in the histological spectrum of angiocentric immunproliferative lesions. This malignancy is commonly a disease with considerable morbidity and is often fatal. However, primary cutaneous angiocentric T-cell lymphoma has a tendency to give a favorable prognosis. Epstein-Barr virus can be identified in involved tissue by immunostaining or by in situ hybridization and it is considered significant in the prognosis. We report a case of cutaneous angiocentric T-cell lymphoma, where the Epstein-Barr virus was identified within neoplastic cells. CVP(cyclophosphamide, vincristine, prednisone) chemotherapy was instigated and an initial complete remission was achieved.
Drug Therapy* ; Herpesvirus 4, Human ; In Situ Hybridization ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Prognosis ; T-Lymphocytes* ; Vincristine

Humans ; Lymphoma, T-Cell, Peripheral ; diagnosis ; therapy ; Translational Medical Research

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (CSMTCL) is a rare disease constituting 2% of all cutaneous T-cell lymphomas. In most cases, CSMTCL occurs as a solitary lesion and has an indolent course. Here we present a 42-year-old woman with multiple, localized, recurrent, erythematous papules and macules that developed after combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP).
Adult ; Cyclophosphamide ; Doxorubicin ; Drug Therapy, Combination ; Female ; Humans ; Lymphoma ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous ; Prednisolone ; Rare Diseases ; T-Lymphocytes ; Vincristine

Objective: To analyze the efficacy and prognostic factors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for treating T lymphoblastic leukemia/lymphoma (T-ALL/LBL) . Methods: This study retrospectively evaluated 119 adolescent and adult patients with T-ALL/LBL from January 2006 to January 2020 at Peking University Third Hospital and Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences. Patients were divided into chemotherapy-only, chemotherapy followed by allo-HSCT, and chemotherapy followed by autologous hematopoietic stem cell transplantation (auto-HSCT) groups according to the consolidation regimen, and the 5-year overall survival (OS) and progression-free survival (PFS) rates of each group were compared. Results: Among 113 patients with effective follow-up, 96 (84.9%) patients achieved overall response (ORR), with 79 (69.9%) having complete response (CR) and 17 (15.0%) having partial response (PR), until July 2022. The analysis of the 96 ORR population revealed that patients without transplantation demonstrated poorer outcomes compared with the allo-HSCT group (5-year OS: 11.4% vs 55.6%, P=0.001; 5-year PFS: 8.9% vs 54.2%, P<0.001). No difference was found in 5-year OS and 5-year PFS between the allo-HSCT and auto-HSCT groups (P=0.271, P=0.197). The same results were achieved in the CR population. Allo-HSCT got better 5-year OS (37.5% vs 0) for the 17 PR cases (P=0.064). Different donor sources did not affect 5-year OS, with sibling of 61.1% vs hap-haploidentical of 63.6% vs unrelated donor of 50.0% (P>0.05). No significant difference was found in the treatment response in the early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP) and non-ETP populations. The ETP group demonstrated lower 5-year OS compared with the non-ETP group in the chemotherapy alone group (0 vs 12.6%, P=0.045), whereas no significant difference was found between the ETP and non-ETP groups in the allo-HSCT group (75.0% vs 62.9%, P=0.852). Multivariate analysis revealed that high serum lactate dehydrogenase level, without transplantation, and no CR after chemotherapy induction were independently associated with inferior outcomes (P<0.05) . Conclusion: Allo-HSCT could be an effective consolidation therapy for adult and adolescent patients with T-ALL/LBL. Different donor sources did not affect survival. Allo-HSCT may overcome the adverse influence of ETP-ALL/LBL on OS.
Adult ; Adolescent ; Humans ; Prognosis ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy* ; Hematopoietic Stem Cell Transplantation ; Lymphoma, T-Cell ; Unrelated Donors

Non-Hodgkin's lymphoma can involve the gynecologic tract, most often as a manifestation of systemic involvement. Primary lymphomas of the female genital tract are rare. Most involve the cervix rather than the uterine corpus. All of those previously reported have been B-cell lymphomas, with the exception of several case reports. Primary T-cell lymphoma of the uterine cervix is rare. We describe a rare case of primary cytotoxic T-cell lymphoma of the uterine cervix, Ann Arbor stage IE in a 55-year-old woman. The tumor, incidentally disclosed at gynecological examination, appeared as diffuse cervical enlargement with mucosal erosion. A biopsy was taken and showed diffuse infiltration of variable sized lymphoma cells. Neoplastic cells were positive for LCA, CD3, TIA-1 and consistent with cytotoxic T-cell lymphoma. She was treated with 3 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone(CHOP). After chemotherapy, the patient was treated by pelvic external radiation therapy. At present, the patient is well and disease-free.
Biopsy ; Cervix Uteri* ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Female ; Gynecological Examination ; Humans ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell* ; Middle Aged ; T-Lymphocytes* ; Vincristine