The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.

SUMMARY OBJECTIVE Describe the clinical and demographic characteristics of pediatric patients with non-Hodgkin’s lymphoma (NHL) enrolled in a tertiary unit of Pediatric Hematology between 1982-2015. PATIENTS AND METHODS A retrospective cohort study of 140 patients aged 16 years or less with NHL. Demographic characteristics, data on diagnosis, and outcomes were analyzed. The overall survival (OS) analysis and stratification by the most frequent histological subtypes were performed using the Kaplan-Meier method. RESULTS One hundred and thirty-six patients with de novo NHL and four with NHL as a second malignancy were analyzed. The median age at diagnosis was 6.4 years (interquartile range, 4.2 to 11.1 years); 101 patients were males. Four patients had primary immunodeficiency, four had human immunodeficiency virus, two post-liver transplantation, and one had autoimmune lymphoproliferative syndrome. The most frequent histological type was NHL of mature B- cell (B-NHL-B; 67.1%), with Burkitt’s lymphoma being the most frequent subtype, and lymphoblastic lymphoma (LBL, 21.4%). The main clinical manifestation at the diagnosis was abdominal tumors (41.4%). During the follow-up time, 13 patients relapsed, but five of them reached a second remission. Thirty-five patients died, and 103 remained alive in clinical remission. No contact was possible for two patients. The OS at 5 years was 74.5% (± 3.8%). The OS estimated for patients with LBL, NHL-B, and the remaining was 80.4%±7.9%, 72.8%±4.7%, and 74.5%±11%, respectively (P = 0.58). CONCLUSION Our results are comparable with cohorts from other middle-income countries.

Objectives: To describe the clinical course of Langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response.Methods: Retrospective analysis of data on 33 children with Langerhans cell histiocytosis followed at Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 1988 and2004. Results: Age at diagnosis ranged from 2 months to 16 years (median: 2.5 years). Seventeen children were male.The follow-up period varied from 21 days to 16.2 years (median: 3.4 years). The most common clinical manifestations at diagnosis were osteolytic lesions, enlarged lymph nodes and skin lesions. The overall survival rate for the whole group was 86.1% at 16 years (95%CI 66.6-94.6%). Deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Those patients who had a "better" response to treatment in the sixth week were likely to have a significantly higher overall survival rate than those who showed disease progression. Overall survival rate was significantly higher for patients with single-system disease. The disease-free survival rate for the whole group was 30.9% at 16 years (95%CI 15.6-47.5%), and was significantly higher for those with single-system disease. Age groups were not associated with different disease-free survival rates. Diabetes insipidus was the most common sequela. No cases of secondary neoplasms were observed. Conclusion:The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.J Pediatr (Rio J). 2007;83(1):79-86: Langerhans cell histiocytosis, diagnosis, therapy, complications.

ObjectivesTo improve the level of 'definitive' diagnosis of Langerhans cell histiocytosis by immunohistochemical investigation of the CD1a surface antigen and to compare outcomes in respect to age, gender, stage of the disease, treatment response and level of diagnostic accuracy.MethodsA retrospective study was carried out of 37 children and adolescents with possible Langerhans cell histiocytosis between 1988 and 2008. The diagnoses were revisited using immunohistochemical investigations for CD1a, S-100 and CD68 in an attempt to reach definitive diagnoses for all cases.ResultsBefore the study, only 13 of 37 patients (35.1%) had a 'definitive' diagnosis; by the end of the study, this number rose to 25 patients (67.6%). All reviewed cases were positive for the CD1a antigen. Overall survival was 88.5%. Multisystem disease (Stage 2; n=19) and absence of response at the 6th week of therapy (n=5) were associated to significantly lower overall survival (p-value = 0.04 and 0.0001, respectively). All deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Other potential prognostic factors were not significant. Reactivation episodes occurred in 75% of the patients with multisystem disease. Diabetes insipidus was the most common sequel (21.6%).ConclusionThe level of diagnostic accuracy was increased through immunohistochemistry. The overall survival rate was similar to international multicentric studies. Multisystem disease and absence of response at six weeks of treatment were the most important unfavorable prognostic factors. The frequency of reactivation for patients with multisystem disease was higher than described in the literature, probably because maintenance chemotherapy was used only in two cases.

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