外周血T细胞淋巴瘤并发骨髓累及的临床病理特征

Mohamed Masoud, C. Xie, Jessica Q Zhou, Xia Chen, M. Xie
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引用次数: 2

摘要

外周T细胞淋巴瘤(PTCLs)不常见,研究较少。大多数ptcl表现为全身性疾病,常累及骨髓。PTCLs累及骨髓可能会损害正常的造血功能,给患者的临床治疗带来更多挑战。本研究对13例淋巴结性外周T细胞淋巴瘤患者的临床病理特征和临床转归进行了分析,并对其进行了骨髓活检。8例患者被诊断为外周T细胞淋巴瘤-非特异性(PTCL-NOS), 3例血管免疫母细胞T细胞淋巴瘤(AITL), 1例间变性大细胞淋巴瘤(ALCL)和1例γ - δ源性T细胞淋巴瘤(tcl - γ / δ)。所有患者外周血异常:贫血11例,血小板减少7例,中性粒细胞减少5例,淋巴细胞增多2例。有趣的是,PTCL的CD4/CD8表型显示与外周血异常相关:CD4+/CD8-表型更常与贫血和血小板减少症相关,CD4-/CD8+表型与淋巴细胞增多症相关。化疗仍然是这些患者进行或不进行干细胞移植的一线治疗的选择。12例患者有治疗和随访资料可供审查,5例患者在随访期间病情缓解或无疾病;5例患者存活或住在安宁疗护中心,但病情持续,2例因多重并发症而死亡。与B细胞淋巴瘤相比,PTCL采用标准化疗的预后较差。多数淋巴结型PTCL患者在初诊时均有骨髓受累,严重影响正常造血功能,可能是导致这些患者整体预后不良的重要因素。进一步的研究和对该病的了解将有助于开发更有效的治疗方法,提高患者的无病生存率和生活质量。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical Pathological Features of Peripheral T Cell Lymphoma with Concurrent Bone Marrow Involvement
Peripheral T cell lymphomas (PTCLs) are uncommon and less well studied. Most PTCLs present as systemic disease and often involve bone marrow. Bone marrow involvement by PTCLs may damage the normal hematopoiesis and bring more challenge to clinical management of these patients. This study focuses on the clinical pathological features and clinical outcomes in 13 patients with nodal peripheral T cell lymphoma and subsequent bone marrow biopsy positive for the same lymphoma. Eight patients were diagnosed of peripheral T cell lymphoma - not otherwise specified (PTCL-NOS), 3 angioimmunoblastic T cell lymphoma (AITL), 1 anaplastic large cell lymphoma (ALCL), and 1 T cell lymphoma of gamma-delta origin (TCL-gamma/delta). All patients had peripheral blood abnormalities: 11 anemia, 7 thrombocytopenia, 5 neutropenia, and 2 lymphocytosis. Of interest, the CD4/CD8 phenotype of PTCL was shown correlating with abnormal peripheral blood findings: CD4+/CD8- phenotype was more often associated with anemia and thrombocytopenia, and CD4-/CD8+ phenotype was associated with lymphocytosis. Chemotherapy remains the choice of first line treatment for these patients with or without stem cell transplantation. 12 patients had treatment and follow up data available for review, 5 were in remission or free of disease during the follow up period; 5 patients were alive or in hospice with persistent disease and 2 died of disease due to multiple complications. The outcome of PTCL treated with the standard chemotherapy has been less favorable compared with B cell lymphomas. Majority of the patients with nodal PTCL have bone marrow involvement at the time of initial diagnosis, which has significant impact on normal hematopoiesis and may be a significant factor in the overall unfavorable prognosis for these patients. Further investigation with better knowledge about this disease will be helpful in the development of more efficient therapy and improve the disease free survival and life quality for these patients.
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