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