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一名年轻女性系统性红斑狼疮伴自身免疫性骨髓纤维化:病例报告

Systemic Lupus Erythematosus Presenting With Autoimmune Myelofibrosis in a Young Woman: A Case Report.

作者信息

Nodeh Mohammad Moeini, Rezaieyazdi Zahra, Karimoddini Motahhareh, Jafarian Amir Hossein, Ghamari Mozhdeh

机构信息

Hematology and Oncology Department, Faculty of Medicine Mashhad University of Medical Sciences Mashhad Iran.

Rheumatic Diseases Research Center Mashhad University of Medical Sciences Mashhad Iran.

出版信息

Clin Case Rep. 2025 Apr 18;13(4):e70362. doi: 10.1002/ccr3.70362. eCollection 2025 Apr.

Abstract

Systemic lupus erythematosus (SLE) is a multifaceted autoimmune disease characterized by different clinical manifestations and often hinders rapid diagnosis. Myelofibrosis, a rare blood disorder characterized by fibrosis replacement in bone marrow and impaired hematopoiesis, is a rare complication of SLE. Case presentation: This report describes a 34-year-old woman who presented with unexplained bicytopenia, initially thought to be attributed to primary myelofibrosis. Subsequent evaluation revealed additional systemic and laboratory findings suggestive of SLE. Her platelet (PLT) count was 7 × 10/μL, platelets increased to 30 × 10/μL after receiving intravenous immunoglobulin (IVIG) and to 75 × 10/μL after receiving Rituximab. The interaction between SLE and bone marrow pathology poses a diagnostic problem and highlights the necessity of comprehensive evaluation when young patients present with unexplained cytopenias. This case emphasizes the necessity of evaluating autoimmune causes such as SLE in patients with myelofibrosis and cytopenia to facilitate rapid diagnosis and appropriate care and thereby prevent consequences.

摘要

系统性红斑狼疮(SLE)是一种多方面的自身免疫性疾病,具有不同的临床表现,常常妨碍快速诊断。骨髓纤维化是一种罕见的血液疾病,其特征是骨髓中出现纤维化替代和造血功能受损,是SLE的一种罕见并发症。病例报告:本报告描述了一名34岁女性,她出现不明原因的双血细胞减少,最初认为是原发性骨髓纤维化所致。随后的评估发现了其他提示SLE的全身和实验室检查结果。她的血小板(PLT)计数为7×10⁹/L,接受静脉注射免疫球蛋白(IVIG)后血小板增加到30×10⁹/L,接受利妥昔单抗后增加到75×10⁹/L。SLE与骨髓病理之间的相互作用带来了诊断难题,并凸显了年轻患者出现不明原因血细胞减少时进行全面评估的必要性。本病例强调了在骨髓纤维化和血细胞减少患者中评估自身免疫性病因如SLE的必要性,以促进快速诊断和适当治疗,从而预防不良后果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3458/12008012/c3b8252a5540/CCR3-13-e70362-g001.jpg

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