关键词: Aplastic anemia Azathioprine Case reports Pancytopenia Systemic lupus erythematosus

Mesh : Humans Anemia, Aplastic / complications diagnosis Pancytopenia / therapy complications Lupus Erythematosus, Systemic / complications diagnosis drug therapy Cyclosporine Cyclophosphamide

来  源:   DOI:10.1007/s00296-024-05585-6

Abstract:
Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus Erythematosus (SLE) patients is extremely rare. The diagnosis may be delayed due to other possible pancytopenia etiologies. Confirmation of peripheral cytopenias diagnosis necessitates a bone marrow aspiration. The management of AA is challenging, and the literature reported using glucocorticoids, danazol, plasmapheresis, cyclophosphamide, intravenous immunoglobulin, and cyclosporine. We report two cases of SLE patients who presented with pancytopenia, with bone marrow biopsy confirmed AA. One case was treated with cyclophosphamide but unfortunately succumbed to Acute Respiratory Distress Syndrome (ARDS), while the other case was managed with rituximab with a good response. Interestingly, both patients were on azathioprine before the diagnosis of AA. A comprehensive search for reported cases of AA in PubMed, Scopus, and the Directory of Open Access Journals databases was performed to enhance the understanding of the diagnostic and management challenges associated with AA in SLE, facilitating ongoing exploration and research in this field. The decision to do a BM aspiration and biopsy is recommended for SLE patients with an abrupt decline in blood counts and previously stable blood counts.
摘要:
再生障碍性贫血(AA)是一种罕见的,潜在的灾难性造血功能衰竭表现为全血细胞减少症和骨髓发育不全。在系统性红斑狼疮(SLE)患者中发生AA极为罕见。由于其他可能的全血细胞减少病因,诊断可能会延迟。外周血细胞减少症的诊断需要骨髓穿刺。机管局的管理具有挑战性,和使用糖皮质激素的文献报道,达那唑,血浆置换,环磷酰胺,静脉注射免疫球蛋白,和环孢菌素.我们报告了两例呈现全血细胞减少症的SLE患者,骨髓活检证实AA。一例接受环磷酰胺治疗,但不幸死于急性呼吸窘迫综合征(ARDS),而另一例采用利妥昔单抗治疗,反应良好。有趣的是,两名患者在诊断AA之前均接受硫唑嘌呤治疗。全面搜索PubMed报告的AA病例,Scopus,并执行了开放获取期刊数据库目录,以增强对与SLE中AA相关的诊断和管理挑战的理解,促进该领域正在进行的探索和研究。对于血液计数突然下降和先前稳定的血液计数的SLE患者,建议决定进行BM抽吸和活检。
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