伴大量浆细胞增殖的血管免疫母细胞性T细胞淋巴瘤临床分析

Clinical analysis of angioimmunoblastic T-cell lymphoma with extreme plasmacytosis

目的:通过对伴大量浆细胞增殖的血管免疫母细胞性T细胞淋巴瘤(AITL)患者进行临床分析,探讨早期诊断的方法。方法:回顾性分析华中科技大学同济医院收治确诊的4例AITL伴大量浆细胞增殖患者的临床特征、影像学资料、病理组织活检、外周血涂片、骨髓细胞学、流式细胞学、分子遗传学、并发症以及治疗和预后。结果:4例患者典型临床表现包括不明原因的发热、肝脾及淋巴结肿大、血清免疫球蛋白显著升高,其中3例患者伴多浆膜腔积液;4例患者的骨髓、外周血和/或胸腹水中有大量浆细胞增殖,甚至类似浆细胞白血病,但流式细胞术检测浆细胞胞浆轻链呈多克隆性表达,免疫球蛋白重链(IgH)重排阴性,进一步验证浆细胞的多克隆性。其中3例患者的预后极差,仅1例存活。结论:伴大量浆细胞增殖的AITL患者,临床表现复杂,需尽早综合各种诊断方法,如组织病理学、流式细胞术、细胞遗传学和分子生物学检查进行精确诊断。

Objective:To discuss early diagnostic methods by investigating the clinical data of angioimmunoblastic T cell lymphoma(AITL)with extreme plasmacytosis.Methods:Totally,4 cases of AITL with extreme plasmacytosis were reported in this study and the clinical features,imaging data,pathological biopsy,peripheral blood smear,bone marrow cytology,flow cytometry,molecular genetics,complications,treatment and prognosis were reviewed.Results:Typical clinical manifestations of these 4 cases of AITL included fever,hepatosplenomegaly and lymphadenopathy and significant increase of globulin in blood.Additionally,polyserous effusions were detected in 3 cases.All 4 patients presented with excessive plasma cell proliferation in bone marrow,peripheral blood,and/or hydrothorax and ascites,even mimicking plasma cell leukemia.The flow cytometry results showed that the cytoplasma light chain of plasma cells was polyclonal,and the immunoglobulin heavy chain(IgH)rearrangement test was negative,which further verified the polyclonality of plasma cells.Three of four patients had poor prognosis and only one patient survived.Conclusion:The clinical manifestations of AITL patients with extreme palsmacytosis are complex.Timely and combined examinations including histopathology,flow cytometry,cytogenetics and molecular biology were required for accurate diagnosis.