Aplastic anemia in neonatal lupus erythematosus
B. Wolach, L. Choc, A. Pomeranz, Y. Ben Ari, D. Douer and A. Metzker
Department of Pediatrics, Meir General Hospital, Sapir Medical Center, Kfar Saba, Israel.
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 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.
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.
