학술논문
Late-onset refractory autoimmune hemolytic anemia following autologous hematologic recovery after allo-HSCT in aplastic anemia-PNH syndrome / 自己造血が回復した同種造血幹細胞移植後の晩期に難治性自己免疫性溶血性貧血を発症した再生不良性貧血-PNH症候群
Document Type
Journal Article
Author
Hisashi SAKAMAKI; Kazuteru OHASHI; Keiichiro HATTORI; Keita YAMAMOTO; Kosuke YOSHIOKA; Naoki SHINGAI; Noriko DOKI; Shinji NAKAO; Shinya ISHIDA; Shuhei KUROSAWA; Takashi TOYA; Takeshi KOBAYASHI; Yasushi SENOO; Yuho NAJIMA; Yutaro HINO; Yuya KISHIDA; 中尾 眞二; 吉岡 康介; 名島 悠峰; 土岐 典子; 坂巻 壽; 大橋 一輝; 妹尾 寧; 小林 武; 山本 圭太; 岸田 侑也; 新谷 直樹; 日野 裕太郎; 服部 圭一朗; 石田 信也; 遠矢 嵩; 黒澤 修兵
Source
臨床血液 / Rinsho Ketsueki. 2022, 63(5):347
Subject
Language
Japanese
ISSN
0485-1439
1882-0824
1882-0824
Abstract
A 31-year-old man underwent allogeneic bone marrow transplantation (BMT) for the treatment of transfusion-dependent aplastic anemia (AA) after conditioning with a regimen including fludarabine, cyclophosphamide, and antithymocyte globulin. The patient developed a late graft rejection on day 103 and showed autologous hematologic recovery not requiring transfusions on day 76. Peripheral blood leukocytes were of 100% recipient origin on day 103, and paroxysmal nocturnal hematuria (PNH)-type granulocytes were detected 5 months after BMT. The patient suddenly experienced hemolytic symptoms triggered by cold stimulation, and was diagnosed with autoimmune hemolytic anemia (AIHA) 37 months after BMT. Although anemia was ameliorated by prednisolone (PSL), hemolytic attacks repeatedly occurred, which became refractory to corticosteroids. Moreover, the patient underwent a splenectomy for the steroid-resistant AIHA and achieved AIHA remission without the need for PSL at 53 months after BMT. The immune tolerance breakdown to erythrocyte antigens was thought to have occurred due to various factors including immune AA, medication, cold stimulation, and infection, leading to AIHA development in this case.