Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
J Pediatr. 1999 Feb;134(2):236-9.

Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance.

Author information

  • 1Service d'Hémato-Immunologie, Centre d'Investigations Cliniques, Laboratoire d'Immunologie, Hôpital Robert-Debré, Paris, France.

Abstract

We describe 4 cases of lysinuric protein intolerance, which all fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis. Mature histiocytes and neutrophil precursors participated in hemophagocytosis in the bone marrow. Moreover, serum levels of ferritin and lactate dehydrogenase were elevated, hypercytokinemia was present, and soluble interleukin-2 receptor levels were increased up to 18.6-fold. The diagnosis of lysinuric protein intolerance should therefore be considered in any patient presenting with hemophagocytic lymphohistiocytosis.

Comment in

PMID:
9931537
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk