Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome
Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome
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DOI：
10.1002/ajh.10258
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发表时间：
2003-01-01
影响因子：
12.8
通讯作者：
Brynes, RK
中科院分区：
文献类型：
--
作者：
Pullarkat, V;Bass, RD;Brynes, RK
骨髓纤维化的特征在于骨髓的网硬蛋白纤维化，导致骨髓增生异常的特征。除了造血系统恶性肿瘤和其他肿瘤涉及骨髓，骨髓纤维化已被描述与自身免疫性疾病，特别是系统性红斑狼疮。我们描述了一种原发性自身免疫性骨髓纤维化（AIMF）的临床病理特征，这些患者没有系统性红斑狼疮或其他明确的自身免疫性综合征。原发性AIMF的一些显著特征是没有明显的脾肿大、外周血细胞减少伴轻度泪滴状异形红细胞增多症和白细胞母细胞增多症、骨髓淋巴样聚集和自身抗体的存在。AIMF应特别与慢性特发性骨髓纤维化（一种肿瘤性骨髓增生性疾病）相鉴别。原发性AIMF的预后似乎很好，本系列报告的所有患者均对短期皮质类固醇治疗有反应。(C)2002 Wiley-Liss，Inc.
Myelofibrosis is characterized by reticulin fibrosis of the bone marrow with resulting features of myelophthisis. Besides hematopoietic malignancies and other neoplasms involving the bone marrow, myelofibrosis has been described in association with autoimmune disorders, especially systemic lupus erythematosus. We describe the clinicopathologic features of a primary form of autoimmune myelofibrosis (AIMF) in patients who do not have systemic lupus erythematosus or another well-defined autoimmune syndrome. Absence of marked splenomegaly, peripheral blood cytopenlas with mild teardrop poikilocytosis and leukoerythroblastosis, bone marrow lymphoid aggregates, and presence of autoantibodies are some of the salient features of primary AIMF. AIMF should especially be differentiated from chronic idiopathic myelofibrosis, a neoplastic myeloproliferative disease. Primary AIMF appears to have an excellent prognosis, with all patients reported in this series responding to a short course of corticosteroid therapy. (C) 2002 Wiley-Liss, Inc.