Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

Daniel Landau*, Evgenia Gurevich, Joseph Kapelushnik, Hannah Tamary, Ilan Shelef, Isaac Lazar

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Background: Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis. Case-diagnosis: We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous complications (many of them reported to accompany NS as single events). Clinical and laboratory signs of HLH evolved with time and led to deterioration of her condition and death, within 5 months of her original presentation. Conclusions: To our knowledge, this is the first report of NS antedating the presentation of HLH.

Original languageEnglish
Pages (from-to)2389-2392
Number of pages4
JournalPediatric Nephrology
Issue number12
StatePublished - Dec 2013


  • Acute kidney injury
  • Hemophagocytic lymphohistiocytosis
  • Macrophage activation syndrome
  • Nephrotic syndrome


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