Neonatal lupus erythematosus with microvascular hemolysis.
A female, term newborn born to a mother with a history of idiopathic thrombocytopenic purpura and antinuclear antibodies, single-stranded A antibody, and IgM anticardiolipin antibodies presented with immune thrombocytopenia, disseminated intravascular coagulation (DIC), microangiopathic hemolytic anemia, and a characteristic lupus rash in the periorbital areas. She responded to combined treatment with dexamethasone and intravenous immunoglobulin (IVIG). At age 9 months, she was readmitted with severe thrombocytopenia, DIC, and microangiopathic hemolytic anemia. She again responded to IVIG. This suggests that microangiopathic hemolysis can be a presenting symptom in neonatal lupus erythematosus and that reoccurrence of the microangiopathic hemolysis may occur even after the disappearance of lupus antibodies.
Hariharan
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Division of Neonatology, The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine 19104, USA.
Manno
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CS
Seri
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D000745:Anemia, Hemolytic, Congenital / Q000276:immunology / Q000628:therapy*; D003907:Dexamethasone / Q000627:therapeutic use; D004211:Disseminated Intravascular Coagulation / Q000151:congenital / Q000276:immunology / Q000628:therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007231:Infant, Newborn; D008180:Lupus Erythematosus, Systemic / Q000151:congenital / Q000276:immunology / Q000628:therapy*; D010949:Plasma; D017713:Platelet Transfusion; D013921:Thrombocytopenia / Q000151:congenital / Q000276:immunology / Q000628:therapy
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D003907:Dexamethasone