Document Detail

Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis.
MedLine Citation:
PMID:  20418784     Owner:  NLM     Status:  MEDLINE    
AIM: To perform a risk factor analysis in patients with "risk organ" multi-system Langerhans cell histiocytosis at diagnosis.
METHODS: From 1987 to 2007, 77 patients were analyzed. A univariate analysis of the variables, age <2 years, lungs, spleen and hepatic involvement, presence of >or=2 risk involved organs, hypoalbuminemia and the presence of isolated anemia, anemia with thrombocytopenia with or without leukopenia at diagnosis was performed. Statistically significant variables were combined and entered into a multivariate analysis.
RESULTS: Fifty-six and 66 evaluable patients had hematologic and hepatic involvement at diagnosis, respectively. Among the hematologic patients, the subgroup of anemia with thrombocytopenia with or without leukopenia showed a significantly lower 5-year survival than the subgroup of isolated anemia (0.19 vs. 0.87, respectively; P=0.0001). Of all the patients, those with hypoalbuminemia had a 5-year survival of 0.16 compared with those with normal albumin levels, who had a 5-year survival of 0.65 (P<0.0001). In multivariate analysis, only anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia were the independent risk factors (relative risk 3.77; confidence interval, 1.7-8.4; P<0.0011 and relative risk 2.59; confidence interval, 1.24-5.4; P<0.0112).
CONCLUSIONS: Anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia, were associated with worse prognosis in multi-system Langerhans cell histiocytosis. Other therapeutic strategies should be considered at diagnosis or early during the initial treatment for this high risk subgroup of patients.
Jorge Luis Braier; Diego Rosso; Antonio Latella; Guillermo Chantada; Blanca Ozuna; Mario Ripoli; Marcelo Scopinaro
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  32     ISSN:  1536-3678     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-05-07     Completed Date:  2010-05-27     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e122-5     Citation Subset:  IM    
Department of Hematology/Oncology, Hospital Nacional de Pediatría Juan P Garrahan, University of Buenos Aires, Buenos Aires, Argentina.
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MeSH Terms
Age Factors
Anemia / complications,  mortality,  pathology*
Cell Lineage*
Child, Preschool
Histiocytosis, Langerhans-Cell / complications,  diagnosis*,  mortality
Hypoalbuminemia / complications,  mortality,  pathology*
Infant, Newborn
Leukopenia / complications,  mortality,  pathology*
Risk Factors
Survival Rate
Thrombocytopenia / complications,  mortality,  pathology*
Treatment Outcome

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