Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis.
OBJECTIVE
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.
Braier
Jorge Luis
JL
Department of Hematology/Oncology, Hospital Nacional de Pediatría Juan P Garrahan, University of Buenos Aires, Buenos Aires, Argentina. jolubraier@gmail.com
Rosso
Diego
D
Latella
Antonio
A
Chantada
Guillermo
G
Ozuna
Blanca
B
Ripoli
Mario
M
Scopinaro
Marcelo
M
D000293:Adolescent; D000367:Age Factors; D000740:Anemia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D019070:Cell Lineage; D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D006801:Humans; D034141:Hypoalbuminemia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D007223:Infant; D007231:Infant, Newborn; D007970:Leukopenia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D008297:Male; D012307:Risk Factors; D015996:Survival Rate; D013921:Thrombocytopenia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D016896:Treatment Outcome