Autoimmune Hemorrhaphilia Resulting from Autoantibody against the A Subunit of Factor XIII

Emi Uchida; Ken Watanabe; Reina Arai; Masahide Yamamoto; Masayoshi Souri; Tsukasa Osaki; Akitada Ichinose; Osamu Miura; Takatoshi Koyama

doi:10.2169/internalmedicine.54.4791

CASE REPORTS

Autoimmune Hemorrhaphilia Resulting from Autoantibody against the A Subunit of Factor XIII

Emi Uchida, Ken Watanabe, Reina Arai, Masahide Yamamoto, Masayoshi Souri, Tsukasa Osaki, Akitada Ichinose, Osamu Miura, Takatoshi Koyama

Author information

Emi Uchida
Department of Hematology, Tokyo Medical and Dental University, Japan
Ken Watanabe
Department of Hematology, Tokyo Medical and Dental University, Japan
Reina Arai
Graduate School of Health Care Sciences, Tokyo Medical and Dental University, Japan
Masahide Yamamoto
Department of Hematology, Tokyo Medical and Dental University, Japan
Masayoshi Souri
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Japan
Tsukasa Osaki
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Japan
Akitada Ichinose
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Japan
Osamu Miura
Department of Hematology, Tokyo Medical and Dental University, Japan
Takatoshi Koyama
Department of Hematology, Tokyo Medical and Dental University, Japan
Graduate School of Health Care Sciences, Tokyo Medical and Dental University, Japan

Keywords: autoimmune hemorrhaphilia, anti-FXIII antibody, A subunit

JOURNAL OPEN ACCESS

2015 Volume 54 Issue 18 Pages 2383-2387

DOI https://doi.org/10.2169/internalmedicine.54.4791

Details

Abstract

A 65-year-old woman was admitted with acute intramuscular hemorrhage of the left gluteus medius and piriformis muscles and associated anemia. Blood tests showed low plasma factor XIII (FXIII) antigen and activity. A cross-mixing test revealed a concave "inhibitor" pattern and anti-FXIII-A subunit antibody was detected. The patient was diagnosed with autoimmune hemorrhaphilia resulting from anti-FXIII antibody. The bleeding has not recurred since the initiation of treatment with oral immunosuppressive agents. Although hemorrhagic acquired FXIII deficiency is a rare disorder, prompt recognition of the underlying mechanism can save lives.

Corresponding author

Register with J-STAGE for free!