title: Aplastic anemia in neonatal lupus erythematosus.
abstract: OBJECTIVE
  To describe an infant with neonatal lupus erythematosus associated with aplastic anemia.
  
  
  METHODS
  The pediatric department in a tertiary-care hospital.
  
  
  METHODS
  Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy.
  
  
  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.
authors.affiliation: Department of Pediatrics, Meir General Hospital, Sapir Medical Center, Kfar Saba, Israel.
authors.forename: B
authors.initials: B
authors.lastname: Wolach
authors.forename: L
authors.initials: L
authors.lastname: Choc
authors.forename: A
authors.initials: A
authors.lastname: Pomeranz
authors.forename: Y
authors.initials: Y
authors.lastname: Ben Ari
authors.forename: D
authors.initials: D
authors.lastname: Douer
authors.forename: A
authors.initials: A
authors.lastname: Metzker
mesh_terms: D000741:Anemia, Aplastic / Q000097:blood / Q000150:complications* / Q000276:immunology; D015672:Erythroid Precursor Cells / Q000737:chemistry; D005260:Female; D006410:Hematopoiesis; D006801:Humans; D007108:Immune Tolerance; D007223:Infant; D008179:Lupus Erythematosus, Discoid / Q000097:blood / Q000150:complications* / Q000276:immunology