COOMBS-NEGATIVE AUTOIMMUNE HEMOLYTIC ANEMIA ASSOCIATED WITH MYELODYSPLASTIC SYNDROME

• Nagai Tadashi

  Division of Hematology, Jichi Medical University Hospital Present address: Central Blood Institute, Japanese Red Cross Society

• Uehara Eisuke

  Division of Hematology, Jichi Medical University Hospital

• Saito Kiriko

  Division of Hematology, Saitama Medical Center, Jichi Medical University

• Kamesaki Toyomi

  Center for Community Medicine, Jichi Medical University Hospital

• Ozawa Keiya

  Research Hospital, The Institute of Medical Science, The University of Tokyo

• Muroi Kazuo

  Division of Cell Transplantation and Transfusion, Jichi Medical University Hospital

• Kanda Yoshinobu

  Division of Hematology, Jichi Medical University Hospital Division of Hematology, Saitama Medical Center, Jichi Medical University

Bibliographic Information

Other Title

• 骨髄異形成症候群に合併したクームス陰性自己免疫性溶血性貧血

Abstract

Myelodysplastic syndrome (MDS) is a hematopoietic disorder characterized by both bone marrow failure and hematopoietic malignancies. It has been shown that autoimmune diseases accompany MDS in 10%-30% of patients. Autoimmune hemolytic anemia (AIHA) is caused by production of autoantibodies against mature erythrocytes, and is diagnosed using the direct Coombs test. Although rare, there are Coombs-negative AIHA patients. In these cases, determination of the level of erythrocyte-associated IgG is critical for diagnosis. Here, we present a case of Coombs-negative AIHA associated with MDS. We treated the patient with the DNA demethylating agent azacitidine for MDS, and consequently, pancytopenia was markedly improved. In addition, myeloblasts in peripheral blood disappeared and the level of Wilms tumor gene mRNA, a marker of MDS progression, became undetectable. Interestingly, during the courses of azacitidine treatment, hemolysis markedly improved, suggesting that azacitidine was also effective in treating AIHA. Coombs-negative AIHA is rare, but it is important to evaluate erythrocyte-associated IgG if the direct Coombs test is negative in patients with hemolytic anemia associated with MDS. Our findings in the present case also suggest the efficacy of azacitidine for AIHA, and thus it is of interest to clarify the mechanism underlying the effect of azacitidine.

Journal

• Japanese Journal of Transfusion and Cell Therapy

  Japanese Journal of Transfusion and Cell Therapy 61 (4), 468-473, 2015

  The Japan Society of Transfusion Medicine and Cell Therapy

Keywords

• myelodysplastic syndrome
• Coombs-negative autoimmune hemolytic anemia
• azacitidine
• erythrocyte-associated IgG

Details

• CRID: 1390001205272485120
• NII Article ID: 130005100034
• DOI: 10.3925/jjtc.61.468
• ISSN: 18830625
• ISSN: 18813011
• Web Site: https://www.jstage.jst.go.jp/article/jjtc/61/4/61_468/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2016062394
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed