伴大颗粒淋巴细胞增多的血管免疫幼细胞性T细胞淋巴瘤

Angioimmunoblastic T-Cell Lymphoma with Large Granular Lymphocytosis

为了提高对血管免疫幼细胞性T细胞淋巴瘤(AITL)的认识,减少临床误诊,本文报告1例伴大颗粒淋巴细胞(LGL)增多的AITL并结合文献进行讨论。对患者进行了一系列临床检查,病理和组织化学检测,T细胞受体基因重排的多重PCR测定及淋巴细胞免疫表型的检查。结果查明:患者有发热、皮疹、全身淋巴结肿大、脾肿大,伴胸水和腹水,贫血和血小板减少,外周血LGL明显增多,免疫表型主要表达CD3-CD16+CD56+,TCRγ基因重排阳性,腹水中LGL易见,有异常核分裂象,淋巴结病理及免疫组织化学检测结果符合AITL,同时伴有CD56阳性表达。结论:AITL多段表现为侵袭性进展,临床预后差,LGL细胞增多可能是机体对肿瘤细胞的免疫反应或来源于肿瘤干细胞。

To improve the recognition of angioimmunoblastic T-cell lymphoma（AITL） and to reduce misdiagnosis,a case diagnosed as AITL with large granular lymphocytosis was reported and the related articles were reviewed.A series of clinical tests,pathologic examination and immunohistochemcal test,TCR gene rearrangement detection by multiple PCR and assay of lymphocyte immunophenotypes were carried out.The results indicated that the patient was characterized by fever,skin rash,generalized lymphadenopathy,splenomegaly,pleural effusion,ascites,anemia and thrombocytopenia,increase of circulating large granular lymphocytes with CD3-and CD16＋,CD56＋ were detected,T-cell recepter γ-chain gene was rearranged.More large granular lymphocytes with abnormal mitosis were found in ascites.The histological and immunohistochemical changes observed by the lymph node biopsy were compatible with AITL,some cells of which were CD56-positive.In conclusion,AITL is characterized by aggressive progress and generally occurs in elderly patients,its clinical prognosis is poor,the large granular lymphocytosis may be an autoimmune response to the tumor cells or originate from tumor stem/progenitor cells.