Leucemia Mieloide Crónica (LMC) Juvenil. A Propósito de un Caso

Autores

Claudio Monge, Esteban Castro, Juan José Rivas-Mejías, Lilliana Calvo, Luisa Vindas-Angulo, María Fernanda Sánchez, Marianela Solano

RESUMEN

La leucemia Mieloide Crónica (LMC) es una neoplasia mieloproliferativa crónica, caracterizada por un gen de fusión t(9,22). El curso normal de la enfermedad consta de 3 fases: crónica, acelerada y blástica. En su gran mayoría los pacientes son diagnosticados en fase crónica y por un hallazgo incidental.  Es común en pacientes mayores de 60 años, y poco frecuentes en niños y adolescentes, de ahí la importancia del reporte de este caso.  Caso clínico: Paciente diagnosticado con LMC que progresó a fase acelerada, debido a falla terapéutica a primera línea de tratamiento con imatibinib y segunda línea con nilotinib.  Dicha falla se justifica por poca adherencia al tratamiento. La importancia de este reporte recae en la baja incidencia de casos de LMC juvenil y las diferencias reportadas en la literatura en cuanto a una progresión clínica más agresiva.

Palabras clave

Leucemia Mielógena Crónica BCR-ABL Positiva, Cromosoma Filadelfia, Mesilato de Imatinib

ABSTRACT

Chronic myeloid leukemia (CML) is a chronic myeloproliferative neoplasm, characterized by a fusion gene t(9,22). The normal course of the disease consists in 3 phases: chronic, accelerated and blastic. The vast majority of patients are diagnosed in the chronic phase by an incidental finding. It is common in patients older than 60 years, and infrequent in children and adolescents, hence the importance of this case report. Clinical case: Patient with a diagnosis of CML that progresses to an accelerated phase, due to therapeutic failure in the first line of treatment with imatibinib and second line with nilotinib. This failure is justified by poor adherence to treatment. This report is important due to the low incidence of cases of juvenile CML and the differences reported in the literature, since it has a more aggressive clinical progression than in adults.

Keywords

Leukemia, Myelogenous, Chronic, BCR-ABL Positive, Philadelphia Chromosome Positive, Imatinib Mesylate
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