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
Department of Hematology, Fukushima Medical University, 1 Hikariga-oka, Fukushima, Fukushima, 960-1295, Japan.
Department of Blood Transfusion and Transplantation Immunology, Fukushima Medical University, Fukushima, Japan.
Int J Hematol. 2017 Nov;106(5):718-724. doi: 10.1007/s12185-017-2268-3. Epub 2017 Jun 5.
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.
我们在此报告一名47岁女性，患有自身免疫性骨髓纤维化（AIMF），伴有自身免疫性肝炎和伊文氏综合征所致的肝损伤。骨髓活检显示骨髓细胞减少，伴有2级网状纤维增生，B淋巴细胞（CD20）、T淋巴细胞（CD3、CD8）和浆细胞（CD138）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）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，使用利妥昔单抗治疗后，患者的全血细胞减少症有所改善，使我们能够迅速降低PSL剂量。本病例提示，骨髓中浸润的淋巴细胞和巨噬细胞中TGF-β表达增加可能与AIMF的发病机制有关。因此，泼尼松龙联合利妥昔单抗可能是治疗激素难治性病例的有效选择。
