Wolach B, Choc L, Pomeranz A, Ben Ari Y, Douer D, Metzker A
Department of Pediatrics, Meir General Hospital, Sapir Medical Center, Kfar Saba, Israel.
Am J Dis Child. 1993 Sep;147(9):941-4. doi: 10.1001/archpedi.1993.02160330031012.
To describe an infant with neonatal lupus erythematosus associated with aplastic anemia.
The pediatric department in a tertiary-care hospital.
Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy.
MEASUREMENTS/MAIN RESULTS: The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8+ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died.
Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.
描述一名患有新生儿红斑狼疮并伴有再生障碍性贫血的婴儿。
一家三级医疗中心的儿科。
输注浓缩红细胞以及为期3周的大剂量类固醇治疗疗程。
测量指标/主要结果：该患者在5个月大时出现严重贫血，面部和颈部有边界清晰的网状斑疹。皮肤病变活检显示表皮角化过度、基底层细胞水肿变性，以及免疫球蛋白和颗粒状C1q在真皮表皮交界处沉积。婴儿体内存在Ro/SS - A抗体。去除抑制性CD8 + T淋巴细胞后，骨髓中的红系祖细胞爆式形成单位（BFU - E）集落增加了约10倍，表明存在对造血的免疫抑制。大剂量类固醇治疗失败。该婴儿随后发展为革兰阴性菌败血症、严重代谢性酸中毒和消耗性凝血病并死亡。
新生儿红斑狼疮可能表现为一系列症状。该病范围可从轻微和短暂到严重、危及生命的状况，需要立即干预，如此处报告的病例。这是首例因免疫介导的造血抑制导致新生儿红斑狼疮合并再生障碍性贫血的报告。
