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
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
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患有自身免疫性肝炎和埃文斯综合征并伴有骨髓中 TGF-β 表达增加的患者的类固醇抵抗性自身免疫性骨髓纤维化：病例报告
DOI：
10.1007/s12185-017-2268-3
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发表时间：
2017
影响因子：
2.1
通讯作者：
Takayuki Ikezoe
中科院分区：
文献类型：
--
作者：
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
我们在此报告一位47岁女性，患有自体免疫性骨髓纤维化（AIMF），并合并有自体免疫性肝炎及伊文思症候群所造成的肝损害。骨髓活检显示骨髓细胞减少伴2级网硬蛋白纤维化，B淋巴细胞（CD 20+）、T淋巴细胞（CD 3+、CD 8+）和浆细胞（CD 138+）水平升高。免疫组织化学分析显示骨髓中浸润的淋巴细胞和巨噬细胞中转化生长因子-β（TGF-β）的表达增加。她最初接受口服泼尼松龙（PSL）治疗2个月，效果有限。然而，在利妥昔单抗治疗后，患者的全血细胞减少症显示出改善，使我们能够迅速减少PSL剂量。本病例提示，骨髓浸润淋巴细胞和巨噬细胞中TGF-β表达增加可能有助于AIMF的发病机制。因此，泼尼松龙联合利妥昔单抗可能是类固醇难治性病例的有效选择。
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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影响因子：
45.3
作者：
Emanuel, Robyn M.;Dueck, Amylou C.;Mesa, Ruben A.
通讯作者：
Mesa, Ruben A.
影响因子：
2.6
作者：
Ungprasert, P.;Chowdhary, V. R.;Makol, A.
通讯作者：
Makol, A.
影响因子：
12.8
作者：
Pullarkat, V;Bass, RD;Brynes, RK
通讯作者：
Brynes, RK
使用多巴胺激动剂治疗围产期心肌病和随后的妊娠取得满意的结果
DOI：
--
发表时间：
2016
期刊：
Revista Brasileira de Ginecologia e Obstetrícia / RBGO Gynecology and Obstetrics
影响因子：
--
作者：
M. A. Melo;Jordão Sousa Carvalho;F. Feitosa;E. Araújo Júnior;A. Peixoto;Francisco Herlânio Costa Carvalho;R. Carvalho
通讯作者：
R. Carvalho
影响因子：
3.3
作者：
Vergara-Lluri, Maria E.;Piatek, Caroline I.;Brynes, Russell K.
通讯作者：
Brynes, Russell K.