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International Journal of Hematology
Published in: International Journal of Hematology 5/2017

01-11-2017 | Case Report

Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report

Authors: Hiroshi Ohkawara, Miki Furukawa, Kazuhiko Ikeda, Akiko Shichishima-Nakamura, Masahiko Fukatsu, Takahiro Sano, Koki Ueda, Satoshi Kimura, Risa Kanai, Yuka Oka, Fumi Murakami, Osamu Suzuki, Yuko Hashimoto, Kazuei Ogawa, Takayuki Ikezoe

Published in: International Journal of Hematology | Issue 5/2017

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Abstract

We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20+), T lymphocytes (CD3+, CD8+), and plasma cells (CD138+). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient’s pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-β in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.
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Metadata
Title
Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report
Authors
Hiroshi Ohkawara
Miki Furukawa
Kazuhiko Ikeda
Akiko Shichishima-Nakamura
Masahiko Fukatsu
Takahiro Sano
Koki Ueda
Satoshi Kimura
Risa Kanai
Yuka Oka
Fumi Murakami
Osamu Suzuki
Yuko Hashimoto
Kazuei Ogawa
Takayuki Ikezoe
Publication date
01-11-2017
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 5/2017
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-017-2268-3

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