Matsuura Minori, Koshiishi Megumi, Suzuki Jun, Suzuki Megumi, Kawashima Ichiro, Nakajima Kei, Kirito Keita
Clinical education center, University of Yamanashi.
Department of Hematology/Oncology, University of Yamanashi.
Rinsho Ketsueki. 2020;61(2):110-115. doi: 10.11406/rinketsu.61.110.
Angioimmunoblastic T-cell lymphoma (AITL) is frequently associated with immunological abnormalities, such as hypergammaglobulinemia, autoimmune cytopenia, and the presence of various autoantibodies. Few reports on AITL have also described the development of myelofibrosis resulting from the invasion of lymphoma cells that produced various cytokines, including TGF-β. Interestingly, recent studies demonstrated that autoimmunity can directly cause autoimmune myelofibrosis (AIMF). Usually, bone marrow fibrosis associated with AIMF is rapidly improved by treatment. Here, we describe our experience with a case of AITL complicated with the presence of numerous autoimmune abnormalities, including positive Coombs, anti-nuclear antibody, anti-ds-DNA antibody, anti-phospholipid antibody, and cold agglutinin tests. The patient presented with severe bone marrow fibrosis (MF-3) at the initial diagnosis. After two courses of the CHASE therapy, myelofibrosis rapidly disappeared, and the autoimmune abnormalities were ameliorated. These findings suggest that the bone marrow fibrosis observed in this case was partly attributable to an AIMF-like mechanism.
血管免疫母细胞性T细胞淋巴瘤（AITL）常与免疫异常相关，如高球蛋白血症、自身免疫性血细胞减少以及各种自身抗体的存在。关于AITL的报道很少，也描述了由产生包括转化生长因子-β在内的各种细胞因子的淋巴瘤细胞浸润导致的骨髓纤维化。有趣的是，最近的研究表明自身免疫可直接导致自身免疫性骨髓纤维化（AIMF）。通常，与AIMF相关的骨髓纤维化通过治疗可迅速改善。在此，我们描述了一例AITL合并多种自身免疫异常的病例，包括库姆斯试验阳性、抗核抗体、抗双链DNA抗体、抗磷脂抗体和冷凝集素试验阳性。患者初诊时表现为严重的骨髓纤维化（MF-3）。经过两个疗程的CHASE治疗后，骨髓纤维化迅速消失，自身免疫异常得到改善。这些发现表明，该病例中观察到的骨髓纤维化部分归因于类似AIMF的机制。
