Nephrotic syndrome accompanying familial hemophagocytic syndrome.
Parole chiave
Astratto
OBJECTIVE
We describe the first reported case of familial hemophagocytic syndrome (FHS) with concurrent minimal change nephrotic syndrome (MCNS).
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.
CONCLUSIONS
This is the first reported case of FHS with coincident MCNS.
