Al-Ghazawi Zaid, Al-Farajat Amwaj, Toubasi Ahmad A, Tawileh Hind B Abu, Qteish Aya, Aladily Tariq N, Alnaimat Fatima
School of Medicine, University of Jordan, Amman, 11942, Jordan.
Department of Pathology, School of Medicine, The University of Jordan, Amman, 11942, Jordan.
Rheumatol Int. 2024 May;44(5):943-953. doi: 10.1007/s00296-024-05585-6. Epub 2024 Mar 21.
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.
再生障碍性贫血(AA)是一种罕见的、潜在灾难性的造血功能衰竭,表现为全血细胞减少和骨髓再生障碍。系统性红斑狼疮(SLE)患者中发生AA极为罕见。由于其他可能导致全血细胞减少的病因,诊断可能会延迟。外周血细胞减少症的诊断确认需要进行骨髓穿刺。AA的治疗具有挑战性,文献报道使用糖皮质激素、达那唑、血浆置换、环磷酰胺、静脉注射免疫球蛋白和环孢素。我们报告了两例SLE患者,他们表现为全血细胞减少,骨髓活检确诊为AA。一例患者接受环磷酰胺治疗,但不幸死于急性呼吸窘迫综合征(ARDS),而另一例患者接受利妥昔单抗治疗,反应良好。有趣的是,两名患者在诊断为AA之前均在服用硫唑嘌呤。我们在PubMed、Scopus和开放获取期刊目录数据库中对已报道的AA病例进行了全面检索,以加深对SLE中与AA相关的诊断和治疗挑战的理解,促进该领域的持续探索和研究。对于血细胞计数突然下降且之前血细胞计数稳定的SLE患者,建议进行骨髓穿刺和活检。