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 Citations (Scopus)

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
StatePublished - May 22 1996

Fingerprint

Hemophagocytic Lymphohistiocytosis
Nephrotic Syndrome
Lipoid Nephrosis
Proteinuria
Biopsy
Hypoalbuminemia
Pancytopenia
Histiocytes
Hypertriglyceridemia
Etoposide
Neutropenia
Thrombocytopenia
Anemia
Siblings
Edema
Adrenal Cortex Hormones
Bone Marrow
Kidney
Therapeutics

Keywords

  • familial hemophagocytic syndrome
  • nephrotic syndrome
  • proteinuria

ASJC Scopus subject areas

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

Cite this

@article{ea03e8fa937a4ffdb441dd0b7185e51f,
title = "Nephrotic syndrome accompanying familial hemophagocytic syndrome",
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.",
keywords = "familial hemophagocytic syndrome, nephrotic syndrome, proteinuria",
author = "Braun, {Michael C.} and Cohn, {Richard A.} and Morris Kletzel",
year = "1996",
month = "5",
day = "22",
doi = "10.1097/00043426-199605000-00021",
language = "English (US)",
volume = "18",
pages = "195--197",
journal = "Journal of Pediatric Hematology/Oncology",
issn = "1077-4114",
publisher = "Lippincott Williams and Wilkins",
number = "2",

}

Nephrotic syndrome accompanying familial hemophagocytic syndrome. / Braun, Michael C.; Cohn, Richard A.; Kletzel, Morris.

In: Journal of pediatric hematology/oncology, Vol. 18, No. 2, 22.05.1996, p. 195-197.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Nephrotic syndrome accompanying familial hemophagocytic syndrome

AU - Braun, Michael C.

AU - Cohn, Richard A.

AU - Kletzel, Morris

PY - 1996/5/22

Y1 - 1996/5/22

N2 - 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.

AB - 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.

KW - familial hemophagocytic syndrome

KW - nephrotic syndrome

KW - proteinuria

UR - http://www.scopus.com/inward/record.url?scp=0029895699&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029895699&partnerID=8YFLogxK

U2 - 10.1097/00043426-199605000-00021

DO - 10.1097/00043426-199605000-00021

M3 - Article

VL - 18

SP - 195

EP - 197

JO - Journal of Pediatric Hematology/Oncology

JF - Journal of Pediatric Hematology/Oncology

SN - 1077-4114

IS - 2

ER -