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目的:提高对肝脾T细胞淋巴瘤诊断和治疗的认识。方法:报道1例罕见肝脾T细胞淋巴瘤并胸膜浸润及转变为淋巴瘤白血病的全过程,并进行文献复习。结果:肝脾T细胞淋巴瘤属外周T细胞淋巴瘤的一种亚型,临床以发热、脾脏显著肿大、肝大为主要表现,淋巴结一般不受累。其病理特征是淋巴瘤细胞的增殖高度局限在肝脾和骨髓的窦内。PCR检测TCRγ或δ基因重排阳性。结论:本病进展快、疗效差,生存期短,可转变为白血病。PCR检测骨髓标本TCRγ基因重排对该病诊断有帮助。对于合并骨髓浸润的肝脾T细胞淋巴瘤应考虑应用白血病的化疗方案,可能会获得较好疗效。
Objective: To improve the diagnosis and treatment of hepatosplenic T-cell lymphoma. Methods: A case of rare hepatosplenic T-cell lymphoma with pleural infiltration and transition to lymphoma leukemia was reported and reviewed. Results: Hepatosplenic T-cell lymphoma is a subtype of peripheral T cell lymphoma. Clinical manifestations include fever, splenic enlargement and hepatomegaly. The lymph nodes are generally not involved. The pathological features of lymphoma cells are highly limited proliferation in the liver and spleen and bone marrow sinus. PCR detection of TCRγ or δ gene rearrangement positive. Conclusion: The rapid progression of the disease, poor efficacy, short survival, can be transformed into leukemia. PCR detection of bone marrow TCRγ gene rearrangement diagnosis of the disease helpful. For the combined marrow infiltration of liver and spleen T-cell lymphoma should consider the application of chemotherapy for leukemia, may get better effect.