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Neonatal lupus erythematosus with microvascular hemolysis
D Hariharan1, C S Manno, I Seri
1Division of Neonatology, The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine 19104, USA.
Journal of Pediatric Hematology/Oncology
|August 26, 2000
Summary
Neonatal lupus erythematosus can present with microangiopathic hemolysis, a serious condition involving red blood cell destruction. This condition may recur even after lupus antibodies are no longer detected.
Area of Science:
- Neonatal immunology
- Pediatric rheumatology
- Hematology
Background:
- Neonatal lupus erythematosus (NLE) is a rare condition caused by transplacental passage of maternal autoantibodies.
- Maternal history included idiopathic thrombocytopenic purpura, antinuclear antibodies, single-stranded A antibody, and IgM anticardiolipin antibodies.
Observation:
- A term newborn presented with immune thrombocytopenia, disseminated intravascular coagulation (DIC), and microangiopathic hemolytic anemia.
- The infant also exhibited a characteristic periorbital lupus rash.
- Initial treatment with dexamethasone and intravenous immunoglobulin (IVIG) led to improvement.
Findings:
- The infant experienced a relapse at 9 months with severe thrombocytopenia, DIC, and microangiopathic hemolytic anemia.
- The patient again responded favorably to IVIG treatment.
- This case highlights microangiopathic hemolysis as a potential presenting symptom of NLE.
Implications:
- Microangiopathic hemolysis can be an initial clinical manifestation of neonatal lupus erythematosus.
- Recurrence of microangiopathic hemolysis in NLE is possible, even without detectable lupus antibodies.
- Early recognition and management of NLE-associated hemolysis are crucial for favorable outcomes.