Paquette R L, Meshkinpour A, Rosen P J
University of California, Los Angeles, School of Medicine.
Medicine (Baltimore). 1994 May;73(3):145-52.
Autoimmune myelofibrosis is an uncommon disorder in which patients present with anemia and thrombocytopenia in conjunction with limited clinical manifestations of autoimmune disease or an exacerbation of previously established SLE. The presence of leukoerythroblastosis in a patient with SLE may suggest the presence of myelofibrosis. Conversely, the absence of splenomegaly in a patient with presumed idiopathic myelofibrosis may suggest an autoimmune etiology. Patients with autoimmune myelofibrosis universally have a positive ANA test and frequently have either elevated anti-DNA titers or a positive LE cell preparation. Because physical manifestations of autoimmune disease may not be evident at presentation, all patients found to have myelofibrosis should have an ANA test. Peripheral blood cytopenias in autoimmune myelofibrosis frequently respond to glucocorticoids but regression of bone marrow fibrosis may be incomplete. Hematologic response to treatment parallels that of the associated autoimmune disease.
自身免疫性骨髓纤维化是一种罕见的疾病，患者会出现贫血和血小板减少，同时伴有自身免疫性疾病的有限临床表现或先前已确诊的系统性红斑狼疮（SLE）病情加重。SLE患者出现幼稚粒-幼红细胞增多可能提示存在骨髓纤维化。相反，疑似特发性骨髓纤维化的患者若没有脾肿大，可能提示自身免疫病因。自身免疫性骨髓纤维化患者的抗核抗体（ANA）检测普遍呈阳性，且经常出现抗DNA滴度升高或狼疮细胞检查阳性。由于自身免疫性疾病的体征在初诊时可能不明显，所有被发现患有骨髓纤维化的患者都应进行ANA检测。自身免疫性骨髓纤维化患者的外周血细胞减少症通常对糖皮质激素有反应，但骨髓纤维化的消退可能不完全。血液学对治疗的反应与相关自身免疫性疾病相似。
