Nephrotic syndrome accompanying familial hemophagocytic syndrome.
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.
Braun
M C
MC
Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA.
Cohn
R A
RA
Kletzel
M
M
D002675:Child, Preschool; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications*; D006801:Humans; D009404:Nephrotic Syndrome / Q000150:complications*