Abstract |
A 37-year-old female who presented with pancytopenia in April 2008 was diagnosed with aplastic anemia stage 2 with a normal karyotype. She had a PNH phenotype in her red blood cells (RBC) and granulocytes, and HLA DR15. Her aplastic anemia was deteriorated from stage 2 to stage 3, and she required periodic RBC transfusions. Four months after cyclosporine therapy, the pancytopenia improved and she did not need RBC transfusion. However, three months thereafter, she again required RBC transfusions after developing severe ulcerative colitis. Although mesalazine and steroid pulse therapy improved her ulcerative colitis, her transfusion dependency persisted. Eleven months after the diagnosis of aplastic anemia, equine anti-thymocyte globulin (ATG) and cyclosporine were administered, but no hematological improvement was obtained. Six months after the administration of ATG and cyclosporine, transformation to refractory cytopenia with multilineage dysplasia (RCMD) with 7-monosomy was observed. An allogeneic bone marrow transplant (BMT) from a HLA-identical sibling was performed 23 months after the diagnosis of aplastic anemia. Complete remission of both the aplastic anemia and ulcerative colitis was obtained without medication. Although the relationship between aplastic anemia and ulcerative colitis remains unclear, immunological abnormalities might be involved in the pathogenesis of both disorders because she had PNH phenotype in RBC and HLA DR15 and because allogeneic BMT improved both disorders.
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Authors | Tsuneyuki Shimada, Tomoya Maeda, Maho Ishikawa, Daisuke Okamura, Yoshihiro Ito, Naoki Wakimoto, Yuichi Nakamura, Nobutaka Kawai, Hirohide Ino, Itsuro Jinnai, Shigehisa Mori, Akira Matsuda, Masami Bessho |
Journal | [Rinsho ketsueki] The Japanese journal of clinical hematology
(Rinsho Ketsueki)
Vol. 53
Issue 2
Pg. 224-8
(Feb 2012)
ISSN: 0485-1439 [Print] Japan |
PMID | 22450583
(Publication Type: Case Reports, English Abstract, Journal Article)
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Chemical References |
- HLA-DR Serological Subtypes
- HLA-DR15 antigen
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Topics |
- Adult
- Anemia, Aplastic
(complications, immunology, therapy)
- Blood Transfusion
- Bone Marrow Transplantation
- Colitis, Ulcerative
(complications, immunology, therapy)
- Female
- HLA-DR Serological Subtypes
- Humans
- Treatment Outcome
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