Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

作者：Landau Daniel^*; Gurevich Evgenia; Kapelushnik Joseph; Tamary Hannah; Shelef Ilan; Lazar Isaac

来源：Pediatric Nephrology, 2013, 28(12): 2389-2392.

DOI：10.1007/s00467-013-2583-8

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

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. %26lt;br%26gt;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. %26lt;br%26gt;To our knowledge, this is the first report of NS antedating the presentation of HLH.

• 出版日期2013-12