ABSTRACT
O presente artigo tem como objetivo conhecer o perfil dos pacientes portadores de Neoplasias Mieloproliferativas Crônicas (NMPC) cromossomo Philadelfia (Ph) negativo clássicas que compreendem as entidades Policitemia Vera (PV), Trombocitose Essencial (TE) e Mielofibrose Primária (MP) na Unidade de Alta Complexidade Oncológica no período de 2008 a 2015. Trata-se de um estudo observacional, descritivo, retrospectivo e de abordagem quantitativa em que foram avaliados 77 prontuários de pacientes com diagnóstico de Policitemia Vera, Trombocitose Essencial e Mielofibrose Primária. Os resultados demonstraram que 54,5% (n=42) tinham diagnóstico de Trombocitose Essencial, 27,3% (n=21) de Policitemia Vera e 18,2% (n=14) de Mielofibrose Primária. A idade média ao diagnóstico girou em torno da sexta e sétima década de vida. As principais alterações laboratoriais encontradas na Policitemia Vera e na Mielofibrose Primária foram a hemoglobina, a qual tem como média 16,05(±2,89) e 9,98(±3,31) respectivamente, e o hematócrito com média de 51,48(±8,69) e 30,70 (±10,14) nesta ordem; já na Trombocitose Essencial as plaquetas foram a principal alteração, com média de 1.728.000(±447136,4). Em relação aos eventos tromboembólicos, 28,6% dos pacientes com PV, 21,4% com TE e 14,3% com MI apresentaram algum tipo de evento (IAM, TVP ou AVC).A mutação JAK2 foi positiva principalmente em portadores de Policitemia Vera, 76,2% (n=16). Dos pacientes portadores de Policitemia Vera 85,7% (n=18), 95,2% (n=40) de Trombocitose Essencial e 57,1% (n=8) de MP permanecem vivos e em tratamento. Concluise que o sexo feminino foi o mais acometido, a idade média ao diagnóstico foi entre a sexta e sétima década de vida, sendo a Trombocitose Essencial a doença mais incidente.
The present article aims to know the profile of patients with classic Philadelphia-negative Chronic Myeloproliferative Neoplasms (CMPNs) who comprehend the entities Polycythemia Vera (PV), Essential Thrombocytosis (ET) and Primary Myelofibrosis (PM) at the High Complexity Oncology Unit between 2008 and 2015. This is an observational, descriptive, retrospective and of quantitative approach study in which 77 diagnosed with Polycythemia Vera, Essential Thrombocytosis and Primary Myelofibrosis patients' charts were analyzed. Results demonstrated that 54,5% (n=42) were diagnosed with Essential Thrombocytosis, 27,3% (n=21) with Polycythemia Vera and 18,2% (n=14) with Primay Myelofibrosis. The mean age at diagnosis was around the sixth and seventh decade of life. The main laboratorial alterations found in Polycythemia Vera and Primary Myelofibrosis were hemoglobin, which has mean 16,05(±2,89) and 9,98(±3,31), respectively, and hematocrit, with mean 51,48(±8,69) and 30,70 (±10,14), in that order; in Essential Thrombocytosis, platelets were the main alteration, with mean 1.728.000(±447136,4). The frequency of thrombotic event was 64,3%, most of which had the diagnosis of Essential Thrombocytosis. The JAK2 mutation was positive mainly on patients with Polycythemia Vera, 76,2% (n=16). 85,7% (n=18) Polycythemia Vera, 95,2% (n=40) Essential Thrombocytosis and 57,1% (n=8) Primary Myelofibrosis patients remain alive and on treatment. It was concluded that females were the most affected, that the mean age at diagnosis was between the sixth and seventh decade of life and that Essential Thrombocytosis was the most incident disease.