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

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...

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Bibliographic Details
Published in:International journal of hematology 2017-11, Vol.106 (5), p.718-724
Main Authors: Ohkawara, Hiroshi, Furukawa, Miki, Ikeda, Kazuhiko, Shichishima-Nakamura, Akiko, Fukatsu, Masahiko, Sano, Takahiro, Ueda, Koki, Kimura, Satoshi, Kanai, Risa, Oka, Yuka, Murakami, Fumi, Suzuki, Osamu, Hashimoto, Yuko, Ogawa, Kazuei, Ikezoe, Takayuki
Format: Article
Language:English
Subjects:
Anemia, Hemolytic, Autoimmune - blood
Anemia, Hemolytic, Autoimmune - complications
Anemia, Hemolytic, Autoimmune - drug therapy
Anemia, Hemolytic, Autoimmune - pathology
Biopsy
Bone marrow
Bone Marrow - metabolism
Bone Marrow - pathology
Case Report
Case reports
CD20 antigen
CD3 antigen
CD8 antigen
Drug Resistance - drug effects
Female
Fibrosis
Hematology
Hepatitis
Hepatitis, Autoimmune - blood
Hepatitis, Autoimmune - complications
Hepatitis, Autoimmune - drug therapy
Hepatitis, Autoimmune - pathology
Humans
Immunotherapy
Liver
Lymphocytes
Lymphocytes B
Lymphocytes T
Macrophages
Medicine
Medicine & Public Health
Middle Aged
Monoclonal antibodies
Myelofibrosis
Oncology
Pancytopenia
Pathogenesis
Plasma cells
Prednisolone
Prednisolone - administration & dosage
Primary Myelofibrosis - blood
Primary Myelofibrosis - complications
Primary Myelofibrosis - drug therapy
Primary Myelofibrosis - pathology
Rituximab
Rituximab - administration & dosage
Thrombocytopenia - blood
Thrombocytopenia - complications
Thrombocytopenia - drug therapy
Thrombocytopenia - pathology
Transforming growth factor
Transforming Growth Factor beta - biosynthesis
Transforming growth factor-b
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Summary: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.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-017-2268-3