September 1993

Aplastic Anemia in Neonatal Lupus Erythematosus

Author Affiliations

From the Department of Pediatrics and the Pediatric Hematology Unit, Meir General Hospital, Sapir Medical Center, Kfar Saba (Drs Wolach, Choc, Pomeranz, and Ben Ari), the Institute of Hematology, Chaim Sheba Medical Center, Tel-Hashomer (Dr Douer), and the Pediatric Dermatology Unit, Children's Medical Center, Petah Tiqva, and the Tel-Aviv University Sackler School of Medicine (Dr Metzker), Israel.

Am J Dis Child. 1993;147(9):941-944. doi:10.1001/archpedi.1993.02160330031012

• 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)