CHẨN ĐOÁN VÀ ĐIỀU TRỊ U LYMPHO THỂ DA NGUYÊN PHÁT TẠI VIỆN HUYẾT HỌC – TRUYỀN MÁU TRUNG ƯƠNG GIAI ĐOẠN 2017-6/2024

Abstract

group of lymphoproliferative diseases, with lymphoproliferative disease that may be confined to the skin without involvement of lymph nodes, bone marrow, or viscera. diagnose. Skin lymphoma can originate from mature T lymphocytes, mature B lymphocytes, or NK cells. Histopathological evaluation including immunohistochemical staining of skin biopsies is the basis of diagnosis, and to date, the treatment of the disease, especially in advanced stages, remains a lot of challenging. Objective: To investigate the characteristics of diagnosis and treatment of primary cutaneous lymphoma at the National Institute of Hematology and Blood Transfusion. Subjects: 58 primary cutaneous lymphoma patients treated at NIHBT from 2017- 6/2024. Methods: Retrospective, descriptive, prospective study. Result: Among the 58 patients included in the study, 79,3% were transferred from Dermatology, while 20,7% came from oncology center. The average time to diagnosis was 19,36 months (ranging from 1 month to 23 years). The mean age of the patients was 50 years (ranging from 7 months to 89 years), with a male-to-female ratio of 1,3:1. All patients (100%) were diagnosed through skin biopsy, with the most common skin lesions being patches and papules (41,4%), followed by nodules (36,2%), tumors (13,8%), and skin ulcers (34,5%). Approximately 25,9% of patients presented with enlarged lymph nodes, and 13.8% had bone marrow infiltration. Among the 44 patients tested, 30,4% were CD30 (+). There were 54 patients with T-cell origin (93,1%), while only 4 patients had B-cell origin (6,9%). Among the 54 patients with T-cell lymphoma, Mycosis fungoides accounted for 29,6%, ALCL CD30 (+) for 5,6%, subcutaneous panniculitis-like T-cell lymphoma for 18,5%, Gamma delta T-cell lymphoma for 3,7%, and peripheral T-cell lymphoma, not otherwise specified (NOS) for 42,6%. In the study group, 56 patients received treatment, with 96,6% undergoing systemic chemotherapy. The treatment regimens included CHOP, CHOPE, CHOPE + lenalidomide, R-CHOP, EPOCH, CVP, GDP, ICE, oral methotrexate, Brentuximab (Adcetris), and pembrolizumab (Keytruda). One patient underwent autologous stem cell transplantation as consolidation after chemotherapy. As of August 2024, 46,5% of the patients had died, 48,3% were still alive, and 5,2% (3 patients) were lost to follow-up. Among the 28 patients who were still alive, 22 patients (78,6%) achieved complete or partial remission, while 6 patients (21,4%) had disease progression. For the 27 patients who died, the average time from diagnosis to death was 46,3 months (ranging from 2 months to 25 years). Conclusion: Patients with primary cutaneous lymphoma are mainly detected from dermatological facilities through skin biopsies, diagnosis and treatment are still a lot of challenging.