Résumé
The association of hematological malignancies with a mediastinal germ cell tumor (GCT) is very rare. We report one case of a young adult male with primary mediastinal GCT who subsequently developed acute megakaryoblastic leukemia involving isochromosome (12p). A 25-yr-old man had been diagnosed with a mediastinal GCT and underwent surgical resection and adjuvant chemotherapy. At 1 week after the last cycle of chemotherapy, his peripheral blood showed leukocytosis with blasts. A bone marrow study confirmed the acute megakaryoblastic leukemia. A cytogenetic study revealed a complex karyotype with i(12p). Although additional chemotherapy was administered, the patient could not attain remission and died of septic shock. This case was definitely distinct from therapy-related secondary leukemia in terms of clinical, morphologic, and cytogenetic features. To our knowledge, this is the first case report of a patient with mediastinal GCT subsequently developing acute megakaryoblastic leukemia involving i(12p) in Korea.
Sujets)
Adulte , Humains , Mâle , Protocoles de polychimiothérapie antinéoplasique/usage thérapeutique , Bléomycine/administration et posologie , Moelle osseuse/anatomopathologie , Chromosomes humains de la paire 12 , Cisplatine/administration et posologie , Étoposide/administration et posologie , Isochromosomes , Caryotypage , Leucémie aigüe mégacaryoblastique/traitement médicamenteux , Tumeurs du médiastin/diagnostic , Tumeurs embryonnaires et germinales/diagnostic , Seconde tumeur primitive/traitement médicamenteux , République de Corée , Choc septique/anatomopathologieRésumé
Os autores fazem a apresentaçäo de um caso de leucemia aguda näo-linfóide em uma criança do sexo feminino, branca, com 27 meses de idade, que foi encaminhada ao St. Jude Children's Research Hospital, com suspeita clínica e radiográfica de neuroblastoma. Esse último diagnóstico foi considerado devido a paciente ser de baixa idade, apresentar dor óssea, anemia, e lesöes osteolíticas disseminadas. Após extensa avaliaçäo, o diagnóstico de leucemia aguda megacarioblástica (LMCA) foi firmado. A definiçäo desse tipo de leucemia, neste caso, se baseou nos achados citomorfológicos do infiltrado celular da medula óssea, e propriedades citoquímicas e imunofenotípicas das células leucêmicas. As características clínicas, biológicas e de resposta ao tratamento da LMCA säo descritas, enfatizando principalmente aquelas que ocorrem em crianças de baixa idade.