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Nephrotic syndrome accompanying familial hemophagocytic syndrome
M C Braun1, R A Cohn, M Kletzel
1Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA.
Purpose:
We describe the first reported case of familial hemophagocytic syndrome (FHS) with concurrent minimal change nephrotic syndrome (MCNS).
Patients And Methods:
This is a case report of a 30-month-old girl who presented to Children's Memorial Hospital with pancytopenia and heavy proteinuria.
Results:
This patient presented with anemia, neutropenia, thrombocytopenia, hypertriglyceridemia, and proteinuria. A brother died at 2 months of age with similar findings. A bone marrow biopsy demonstrated histiocyte proliferation with marked erythrophagocytosis, consistent with FHS. Treatment was begun with corticosteroids and VP-16. The patient developed worsening peripheral edema and hypoalbuminemia, with heavy proteinuria. After 1 month of therapy with persistence of heavy proteinuria, a renal biopsy was performed, the results of which were consistent with MCNS.
Conclusion:
This is the first reported case of FHS with coincident MCNS.