Cytogenetic abnormalities, WHO classification, and evolution of children and adolescents with acute myeloid leukemia

ABSTRACT Objectives: To describe cytogenetic and molecular abnormalities observed in children and adolescents with acute myeloid leukemia (AML), classify AML according to the World Health Organization (WHO) classifications from 2008 and 2016, and evaluate the prognosis according to clinical characteristics and cytogenetic abnormalities. Methods: A retrospective longitudinal study was performed on a population of 98 patients with AML, aged up to 16 years, seen in a single hospital from 2004 to 2015. Results: Among the 80 patients for whom it was possible to analyze the karyotype, 78.7% had chromosomal changes, the most frequent being t(15;17)(q22;q21). Of the 86 patients for whom we had cytogenetic or molecular data, making it possible to classify their AML according to the WHO classification, 52.3% belonged to the group with recurrent genetic abnormalities, 22% to the "AML not otherwise specified" group, 18.6% to the group with myelodysplasia-related cytogenetic changes, and 7% to the group with Down syndrome-related leukemia. Five-year overall survival (OS) for the whole group was 49.7% ± 5.2%. In the univariate and multivariate analyses, patients with myelodysplasia-related cytogenetic changes (OS 28.1% ± 12.2%) and those with "AML not otherwise specified" (OS 36.1% ± 11.2%) had an unfavorable prognosis when compared to patients with AML with recurrent genetic abnormalities (OS 71% ± 5.8%) and patients with Down syndrome-related AML (OS 83% ± 15.2%, p = 0.011). Conclusions: The results corroborate the importance of cytogenetic abnormalities as a prognostic factor and indicate the need for cooperative and prospective studies to evaluate the applicability of the WHO classification in the pediatric population.

Anemia hemolítica auto-imune como sintoma inicial de linfoma de hodgkin / Autoimmune hemolytic anemia as an initial symptom in Hodgkin's disease

Objetivo: relatar um caso de linfoma de Hodgkin precedido por anemia hemolítica auto-imune (AHAI). Descrição: relata-se o caso de um paciente de 10 anos de idade com quadro clínico e laboratorial típico de AHAI por anticorpo de reação fria. Após quatro anos de evolução, com resposta inicial ao uso de corticóide e imunoglobulina, o paciente apresentou anemia refratária, hepatoesplenomegalia, febre e perda de peso, sendo diagnosticado linfoma de Hodgkin (LH) estádio IIB. Após 23 meses de encerramento da quimioterapia, o paciente encontra-se em remissão clínica, tanto da AHAI quanto do linfoma. Comentários: a AHAI consiste de um grupo de doenças cuja característica comum é a presença de auto-anticorpos que diminuem o tempo de sobre vida dos eritrócitos. A maioria dos casos de AHAI crônica é secundária a uma doença de base grave, assim, esses pacientes demandam seguimento clínico rigoroso e prolongado. O presente relato de caso mostra que o LH deve ser considerado no diagnóstico diferencial de uma criança com AHAI crônica.