白血病阶段的小细胞亚型间变性大细胞淋巴瘤1例并文献复习

A case report of small cell variant of anaplastic large cell lymphoma in leukemic phase and literature review

目的探讨间变性大细胞淋巴瘤（ALCL）白血病阶段的临床特点、诊断、治疗及相关进展。方法对一例白血病阶段的ALK阳性的ALCL患者的诊治经过进行回顾性分析及相关文献复习。结果4岁男童因淋巴结肿大24d入院，院外按淋巴结炎抗感染治疗无效。入院时患儿高热，精神差，肝、脾、淋巴结肿大，白细胞81．99×10^9／L。根据病理、FISH、骨髓流式细胞术、细胞因子检查确诊为小细胞型ALCL的白血病阶段，合并高细胞因子血症。给予血浆置换使患者一般状态得到改善后立即给予化疗，期间合并骨髓抑制及感染，家长放弃治疗。结论小细胞病理亚型的ALCL侵袭性强，可以合并白血病，易和感染性疾病及其他小细胞肿瘤相混淆，需引起临床医生的注意。患者可合并高细胞因子血症，致病情危重，治疗风险大。

Objective To investigate the clinical manifestation, diagnosis, treatment of ALK positive anaplastic large cell lymphoma （ALCL）in leukemic phase. Methods We collected an ALCL leukemia patient＇ s clinical materials retrospectively to analyze the clinical features and review the related references. Results The patient is a 4 years old boy. He was admitted in our hospital with a complaint of ＂cervical adenopathy for 24 days ＂ He received anti-infective treatment before coming to our hospital, without any improvement. He had a high fever, fatigue, cervical lymph nodes enlargement, hepatosplenomegaly. Leukocyte count was 81.99 ×10^9/L, IL-2,IL-4,IFN,TNF level was much higher than normal. According to the outcomes of pathology, FISH, flow cytometry, he was diagnosed as small variant ALCL in leukemic phase. His condition was improved by plasmapheresis and chemotherapy, but he was complicated with serious bone marrow suppression and infection. His parents gave up treatment then. Conclusions Leukemic involvement in ALCL is often associated with the small cell histologic features. They are easily misdiagnosed as infective diseases and other tumors composed of small cells. Some patients are complicated with cytokines storm, the mortality rate is high.