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Aplastic Anemia in Neonatal Lupus Erythematosus

Baruch Wolach, MD; Luis Choc, MD; Avshalom Pomeranz, MD; Yosef Ben Ari, MD; Danny Douer, MD; Aryeh Metzker, MD
Am J Dis Child. 1993;147(9):941-944. doi:10.1001/archpedi.1993.02160330031012.
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• Objective.  —To describe an infant with neonatal lupus erythematosus associated with aplastic anemia.

Setting.  —The pediatric department in a tertiary-care hospital.

Interventions.  —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 C1 q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythyroid 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.

Conclusions.  —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.(AJDC. 1993;147:941-944)


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