Pullarkat Vinod, Bass Randall D, Gong Jerald Z, Feinstein Donald I, Brynes Russell K
Division of Hematology, Keck School of Medicine, University of Southern California, Los Angeles, California 91010-300, USA.
Am J Hematol. 2003 Jan;72(1):8-12. doi: 10.1002/ajh.10258.
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 cytopenias 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.
骨髓纤维化的特征是骨髓网状纤维纤维化，并伴有骨髓痨的相关特征。除了造血系统恶性肿瘤和其他累及骨髓的肿瘤外，骨髓纤维化还被描述与自身免疫性疾病有关，尤其是系统性红斑狼疮。我们描述了在无系统性红斑狼疮或其他明确自身免疫综合征患者中一种原发性自身免疫性骨髓纤维化（AIMF）的临床病理特征。无明显脾肿大、外周血细胞减少伴轻度泪滴状异形红细胞增多和幼粒幼红细胞血象、骨髓淋巴样聚集以及自身抗体的存在是原发性AIMF的一些显著特征。AIMF尤其应与慢性特发性骨髓纤维化（一种肿瘤性骨髓增殖性疾病）相鉴别。原发性AIMF似乎预后良好，本系列报道的所有患者对短期皮质类固醇治疗均有反应。
