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间变大细胞淋巴瘤（ALCL）是一种临床上少见的恶性淋巴瘤。形态学存在着一种标志的hall mark细胞；细胞免疫学上 CD30(Ki-1)表达强阳性；标志性的染色体异常t(2；5)(p23；q35)。临床诊断时多为疾病晚期(Ⅲ～Ⅳ期)，并常伴有B 症状。成人患者，推荐采取放疗，同时辅以联合化疗;儿童患者病程类似Burkitt 淋巴瘤，治疗方案一般使用高强度的治疗淋巴母细胞白血病或 Burkitti 淋巴瘤的方案。高危ALCL、复发难治ALCL 可应用CD30单克隆抗体和ALK蛋白抑制剂等。临床上报道较少，对其认识仍有不足。近年来虽然对ALCL 的研究取得了显著的进展，但至今尚未形成成熟规范的治疗方案。未来研究有望最终寻找到 ALCL的最优化治疗方案。现就其在免疫和遗传学、形态学、临床表现、治疗和预后等方面的研究进展作一综述。

英文摘要:

Anaplasia large cell lymphoma (ALCL) is a clinically rare malignant lymphoma. There is a symbol existence, it is the hall mark cells; CD30 (Ki - 1) is strongly expressed on Cells. The most frequent genetic alter-ation is a translocation, t (2;5)(p23;q35). Clinical diagnosis of more advanced disease (Ⅲ ~Ⅳ stage), and is often accompanied by B symptoms. The current ALCL treatment is based on radiotherapy combination chemotherapy in adult patients. Children patients with similar to the Burkitt lymphoma, treatment is generally used in the treatment of high strength lymphatic mother cell leukemia or lymphoma Burkitti program. High-risk ALCL, recurrence obstinacy ALCL can be used CD30 monoclonal antibody and ALK protein inhibitors, etc. Clinical reports less, the understanding is still insufficient. In recent years, although the research of ALCL made significant progress, but have not mature standard treatments. Future study is expected to finally find the ALCL optimization treatment .Now reviewed its progress in the study of immunity and genetics, morphology, clinical manifestations, treatment and prognosis.

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