Xu Juan, Tang Yuan, Zhao Sha, Zhang WenYan, Xiu YingJie, Liu Ting, Wu Yu
Department of Hematology and Hematology Research Laboratory, West China Hospital, Sichuan University.
Tohoku J Exp Med. 2015 Apr;235(4):283-8. doi: 10.1620/tjem.235.283.
Angioimmunoblastic T-cell lymphoma (AITL) is recognized as a distinct clinicopathological subtype of peripheral T-cell lymphomas. Its clinical features include generalized lymphadenopathy, constitutional symptoms, and autoimmune-related findings, such as hemolytic anemia. Pathologically, AITL is characterized by a polymorphous infiltrate in lymph nodes with prominent proliferation of high endothelial venules and follicular dendritic cells. We present an 80-year-old Chinese man with generalized lymphadenopathy and pulmonary infection, diagnosed as AITL based on the distinctive pathological findings and T-cell receptor gamma (TCR-γ) gene rearrangement analysis of lymph nodes. Importantly, the patient suffered from a coexisting plasma cell myeloma, as judged by monoclonal immunoglobulin in the serum, immature plasma cells, and rearrangement of the immunoglobulin heavy-chain (IgH) gene in the bone marrow. The patient received two courses of the chemotherapy but died of pneumonia 6 months after diagnosis. AITL can be accompanied by polyclonal or clonal proliferation of B lymphocytes; however, AITL are rarely associated with plasma cell proliferation. In fact, 14 AITL cases with plasma cell proliferation have been reported in the literature, but none of them manifested the infiltration of monoclonal immature plasma cells in the bone marrow. To the best of our knowledge, this is the first report of newly diagnosed, concurrent AITL and plasma cell myeloma, providing the evidence for the interplay between malignant T cells and plasma cell proliferation. A review of the literature has also supported a relationship between AITL and plasma cell proliferation. Awareness of this relationship is important for correct diagnosis and appropriate treatment of AITL.
血管免疫母细胞性T细胞淋巴瘤(AITL)被认为是外周T细胞淋巴瘤的一种独特的临床病理亚型。其临床特征包括全身淋巴结肿大、全身症状以及自身免疫相关表现,如溶血性贫血。病理上,AITL的特征是淋巴结内多形性浸润,伴有高内皮小静脉和滤泡树突状细胞的显著增殖。我们报告一例80岁中国男性,有全身淋巴结肿大和肺部感染,根据独特的病理表现及淋巴结的T细胞受体γ(TCR-γ)基因重排分析诊断为AITL。重要的是,根据血清中单克隆免疫球蛋白、未成熟浆细胞以及骨髓中免疫球蛋白重链(IgH)基因重排判断,该患者同时患有浆细胞骨髓瘤。患者接受了两个疗程的化疗,但在诊断后6个月死于肺炎。AITL可伴有B淋巴细胞的多克隆或克隆性增殖;然而,AITL很少与浆细胞增殖相关。事实上,文献中已报道14例伴有浆细胞增殖的AITL病例,但均未表现出骨髓中单克隆未成熟浆细胞浸润。据我们所知,这是首例新诊断的同时并发AITL和浆细胞骨髓瘤的报告,为恶性T细胞与浆细胞增殖之间的相互作用提供了证据。文献回顾也支持AITL与浆细胞增殖之间的关系。认识到这种关系对于AITL的正确诊断和恰当治疗很重要。