Document Detail


Hemophagocytic Lymphohistiocytosis--A Diagnostic Dilemma: Two Cases and Review.
MedLine Citation:
PMID:  23172902     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory disorder characterized by activation and proliferation of lymphocytes and histiocytes with cytokine release and uncontrolled hemophagocytosis, especially late in the course of the disease. Clinical features include relapsing fevers, hepatosplenomegaly, cytopenias, lymphadenopathy, and coagulopathy. The diagnosis can be challenging, as the early signs and symptoms are nonspecific and no specific laboratory tests exist. This syndrome is frequently not recognized and has a significant mortality rate. Typical scenarios in which HLH should be considered include mononucleosis (fever, hepatosplenomegaly, and lymphadenopathy) in an infant or young child, aseptic meningitis associated with cytopenias, or a viral syndrome-like illness with cytopenias and lymphadenopathy or splenomegaly, for example. Our approach includes measuring a ferritin level as a screening tool early in the course of such an illness. Two cases of HLH are reviewed, illustrating the frequent complexity of these cases and potential pitfalls to making a prompt diagnosis.
Authors:
Aarti Bhasin; Robert W Tolan
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-19
Journal Detail:
Title:  Clinical pediatrics     Volume:  -     ISSN:  1938-2707     ISO Abbreviation:  Clin Pediatr (Phila)     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372606     Medline TA:  Clin Pediatr (Phila)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
1Saint Peter's University Hospital, New Brunswick, NJ, USA.
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