Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis.

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.

Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis.

PURPOSE: Langerhans cell histiocytosis (LCH) is a rare disease with variable prognosis in which lesions and clinical features suggest that pro- and anti-inflammatory cytokines may be involved in its pathogenesis. The authors wished to evaluate whether serum levels of interleukin-1 receptor agonist (IL-1Ra) and tumor necrosis factor-alpha (TNF-alpha) are elevated in children with LCH and decrease after chemotherapy. PATIENTS AND METHODS: Circulating levels of IL-1Ra and TNF-alpha were measured in 23 and 8 children with LCH, respectively, and 7 pediatric controls using commercially available ELISA kits. All patients fulfilled the Histiocyte Society LCH Protocols criteria for diagnosis, stratification, and treatment. RESULTS: Pretreatment concentrations of IL-1Ra and TNF-alpha were found to be significantly elevated in patients with LCH compared with controls. Among LCH substages, a significant difference in IL-1Ra values was observed between individuals with single-system single-site disease vs. multisystem disease with risk-organ dysfunction. In all eight patients evaluated, IL-1Ra levels decreased after 6 weeks of chemotherapy. Lower values of TNF were observed in three patients after treatment. A positive and significant correlation between IL-1Ra and TNF serum concentrations was found. CONCLUSIONS: Patients with LCH have elevated levels of IL-1Ra and TNF, which decrease after chemotherapy.

Histiocitosis / Hystiocitosis

Se denomina Histiocitosis a un grupo heterogéneo de alteraciones del sistema monocítico-macrofágico. Este se halla constituido por elementos que provienen de los promonocitos de la médula ósea y que dan origen a dos líneas celulares: 1) célula procesadora de antígenos, representada por el monocito sanguíneo que da lugar al macrófago tisular, 2) célula presentadora de antígenos, que incluye 3 subgrupos celulares: reticulares dendríticas, reticulares interdigitantes y de Langerhans. Cada síndrome histiocítico es el resultado de la proliferación reactiva o neoplásica de uno de estos tipos celulares. En 1987 la Sociedad del Histiocito recomendó dividir los sindromes histiocíticos en tres clases: Clase I- Histiocitosis de células de Langerhans (presentadoras de antígeno) y Reticulohistiocitosis congénita autoinvolutiva de Hashimoto-Pritzker. Clase II- Histiocitosis de fagocitos mononucleares (procesadoras de antígeno). Clase III- Histiocitosis malignas. En esta actualización haremos especial referencia a los sindromes histiocíticos de la Clase I basados en nuestra experiencia desde Octubre de 1987 a Mayo de 1994

Histiocitosis / Hystiocitosis

Se denomina Histiocitosis a un grupo heterogéneo de alteraciones del sistema monocítico-macrofágico. Este se halla constituido por elementos que provienen de los promonocitos de la médula ósea y que dan origen a dos líneas celulares: 1) célula procesadora de antígenos, representada por el monocito sanguíneo que da lugar al macrófago tisular, 2) célula presentadora de antígenos, que incluye 3 subgrupos celulares: reticulares dendríticas, reticulares interdigitantes y de Langerhans. Cada síndrome histiocítico es el resultado de la proliferación reactiva o neoplásica de uno de estos tipos celulares. En 1987 la Sociedad del Histiocito recomendó dividir los sindromes histiocíticos en tres clases: Clase I- Histiocitosis de células de Langerhans (presentadoras de antígeno) y Reticulohistiocitosis congénita autoinvolutiva de Hashimoto-Pritzker. Clase II- Histiocitosis de fagocitos mononucleares (procesadoras de antígeno). Clase III- Histiocitosis malignas. En esta actualización haremos especial referencia a los sindromes histiocíticos de la Clase I basados en nuestra experiencia desde Octubre de 1987 a Mayo de 1994