NEPHROTIC SYNDROME ACCOMPANYING FAMILIAL HEMOPHAGOCYTIC SYNDROME

Purpose: We describe the first reported case of familial hemophagocyti c syndrome (FHS) with concurrent minimal change nephrotic syndrome (MC NS). Patients and Methods: This is a case report of a 30-month-old gir l who presented to Children's Memorial Hospital with pancytopenia and heavy proteinuria. Results: This patient presented with anemia, neutro penia, thrombocytopenia, hypertriglyceridemia, and proteinuria. A brot her died at 2 months of age with similar findings. A bone marrow biops y demonstrated histiocyte proliferation with marked erythrophagocytosi s, consistent with FHS. Treatment was begun with corticosteroids and V P-16. The patient developed worsening peripheral edema and hypoalbumin emia, with heavy proteinuria. After 1 month of therapy with persistenc e of heavy proteinuria, a renal biopsy was performed, the results of w hich were consistent with MCNS. Conclusion: This is the first reported case of FHS with coincident MCNS.