Nephrotic syndrome accompanying familial hemophagocytic syndrome

Michael C. Braun, Richard A. Cohn, Morris Kletzel*

*Corresponding author for this work

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

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 ith 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.

Original languageEnglish (US)
Pages (from-to)195-197
Number of pages3
JournalJournal of pediatric hematology/oncology
Volume18
Issue number2
DOIs
Publication statusPublished - May 22 1996

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Keywords

  • familial hemophagocytic syndrome
  • nephrotic syndrome
  • proteinuria

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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