Pundole Xerxes, Konoplev Sergej, Oo Thein Hlaing, Lu Huifang
From the Section of Rheumatology, Department of General Internal Medicine (XP,HL); Department of Hematopathology, Division of Pathology/Lab Medicine (SK) and Section of Benign Hematology, Division of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA (THO).
Medicine (Baltimore). 2015 May;94(19):e741. doi: 10.1097/MD.0000000000000741.
Autoimmune myelofibrosis is a distinct clinicopathologic entity that occasionally occurs with autoimmune disorders such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Most cases of autoimmune myelofibrosis have been reported in female patients with a known history of SLE. We report a case of a middle-aged male patient with an unusual presentation of SLE and autoimmune myelofibrosis who presented only with severe anemia initially and was later diagnosed with SLE and autoimmune myelofibrosis. The patient's condition improved dramatically after treatment with corticosteroids.SLE and autoimmune myelofibrosis is a rare but potentially devastating condition. Anemia maybe the only presenting symptom in addition to bone marrow fibrosis and careful clinical and laboratory assessment is imperative. Corticosteroids maybe useful and spare patients from bone marrow transplantation.
自身免疫性骨髓纤维化是一种独特的临床病理实体，偶尔会与自身免疫性疾病如系统性红斑狼疮（SLE）和类风湿性关节炎同时出现。大多数自身免疫性骨髓纤维化病例报告见于有SLE病史的女性患者。我们报告一例中年男性患者，其SLE和自身免疫性骨髓纤维化表现异常，最初仅表现为严重贫血，后来被诊断为SLE和自身免疫性骨髓纤维化。患者经皮质类固醇治疗后病情显著改善。SLE和自身免疫性骨髓纤维化是一种罕见但可能具有毁灭性的疾病。除骨髓纤维化外，贫血可能是唯一的表现症状，因此必须进行仔细的临床和实验室评估。皮质类固醇可能有用，可使患者免于骨髓移植。
