Nozaki Yumi, Koshiishi Megumi, Sueki Yuki, Kawashima Ichirou, Yamamoto Takeo, Nakajima Kei, Mitsumori Toru, Kirito Keita
Department of Hematology and Oncology, University of Yamanashi.
Rinsho Ketsueki. 2015 Mar;56(3):317-22. doi: 10.11406/rinketsu.56.317.
A 60-year-old woman was admitted to our hospital with anemia and thrombocytopenia. Serum testing showed platelet-associated IgG elevation and she was positive on the direct and indirect Coombs tests. Together with bone marrow examination, these findings indicated a diagnosis of Evans syndrome. At diagnosis, she also had an IgM-κ type of monoclonal gammopathy of unknown significance. Initially, we administered steroids and her hemolytic anemia showed improvement. In contrast, only transient recovery of platelet counts was observed and her platelet counts rapidly decreased after steroid dose reduction. Thus, we treated her with a TPO-agonist, romiplostim. During the clinical course, she showed gradual serum IgM elevation. We thus performed another bone marrow biopsy and diagnosed her as having Waldenström's macroglobulinemia (WM). We started treatment with rituximab for WM. Together with the serum IgM reduction, she showed marked improvement of thrombocytopenia. This is a very rare case of WM initially presenting as autoimmune hemolytic anemia and immunethrombocytopenia associated with IgG class auto-antibody. Our experience suggests the usefulness of rituximab and romiplostim for the treatment of immunethrombocytopenia associated with WM.
一名60岁女性因贫血和血小板减少症入住我院。血清检测显示血小板相关IgG升高，直接和间接抗人球蛋白试验均为阳性。结合骨髓检查，这些结果提示诊断为伊文氏综合征。诊断时，她还患有意义未明的IgM-κ型单克隆丙种球蛋白病。起初，我们给予类固醇治疗，她的溶血性贫血有所改善。相比之下，仅观察到血小板计数短暂恢复，类固醇剂量减少后血小板计数迅速下降。因此，我们用促血小板生成素激动剂罗米司亭对她进行治疗。在临床过程中，她的血清IgM逐渐升高。于是我们再次进行骨髓活检，诊断她患有华氏巨球蛋白血症（WM）。我们开始用利妥昔单抗治疗WM。随着血清IgM降低，她的血小板减少症有明显改善。这是一例非常罕见的WM病例，最初表现为自身免疫性溶血性贫血和与IgG类自身抗体相关的免疫性血小板减少症。我们的经验表明，利妥昔单抗和罗米司亭对治疗与WM相关的免疫性血小板减少症有用。
