自身免疫性骨髓纤维化：系统性红斑狼疮中一种罕见的血液系统受累情况。
Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus.
作者信息
Belfeki Nabil, Shankarasivam Gopinath, Declerck Damienne, Diamantis Sylvain
机构信息
Internal Medicine and Infectious Disease Department, Centre Hospitalier Sud Ile de France, Melun, France.
Pathological Laboratory, Laboratoire d'anatomopathologie d'Avon, Avon, France.
出版信息
BMJ Case Rep. 2019 Jan 14;12(1):bcr-2018-227520. doi: 10.1136/bcr-2018-227520.
Autoimmune myelofibrosis is a distinct clinicopathological entity that occurs with autoimmune disorders. We report the case of a 44-year-old woman admitted with pancytopenia and clinical features of systemic lupus erythematosus, Sjogren's syndrome and antiphospholipid antibodies syndrome. Bone marrow biopsy showed decreased global cells and an increase of reticulin fibres on argentic coloration, consistent with myelofibrosis. The JAK2 V617, Myeloproliferative leukemia (MPL) and calreticulin mutations were negative. The patient's condition improved after treatment with hydroxychloroquine, vitamin K antagonists and prednisone.
自身免疫性骨髓纤维化是一种与自身免疫性疾病相关的独特临床病理实体。我们报告了一例44岁女性患者，因全血细胞减少入院，具有系统性红斑狼疮、干燥综合征和抗磷脂抗体综合征的临床特征。骨髓活检显示细胞总数减少，银染色显示网状纤维增多，符合骨髓纤维化表现。JAK2 V617、骨髓增殖性白血病（MPL）和钙网蛋白突变均为阴性。患者经羟氯喹、维生素K拮抗剂和泼尼松治疗后病情改善。
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