Síndrome Mieloproliferativa Crônica Inclassificável: Relato de Caso/Nondescript Chronicles Myeloproliferative Syndrome: Case Report
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Abstract
Introdução: As neoplasias mieloproliferativas são patologias que se originam de células tronco totipotentes e se caracterizam por multiplicação acelerada de uma ou mais séries sanguíneas, sem relevante displasia. São classificadas em diversos subtipos entre eles: Leucemia Mielóide Crônica, Leucemia Neutrofílica Crônica,Mielofribrose Primária e Doença Mieloproliferativa Crônica Inclassificável. Essa classificação justifica-se por mutações genéticas que definem a evolução e a fisiopatologia de cada doença. Casuística: Apresentamos o caso de uma paciente, sexo feminino, 58 anos, com queixas inespecíficas, que por achado laboratorial suspeitou-se de leucemia. Foi submetida à investigação clínica, laboratorial,radiológica, e realizada a biópsia de MO, sendo diagnosticada com Síndrome Mieloproliferativa Crônica Inclassificável. Foi orientada a fazer uso de hidroxiureiapara controle hematimétrico, porém sem melhora, iniciou citarabina. Paciente encontra-se na fase blástica da doença e devido à ausência de remissão da patologia,foi encaminhada para lista de transplante. Discussão: o caso foi bem conduzido quanto aos métodos diagnósticos utilizados na investigação da mesma, sendo fundamental o RT-PCR para identificação do cromossomo Philadelphia. O tratamento foi semelhante à literatura em relação aos medicamentos usados, porém algumas posologias não foram as mesmas. A condução do caso levou a paciente paraa fila de transplante, visto que não houve resposta terapêutica. Conclusão: este relato de caso trata-se, portanto, de uma Neoplasia Mieloproliferativa Crônica do tipo inclassificável, pois não preenche os critérios diagnósticos preconizados pela Organização Mundial de Saúde, visto que foi classificada como Leucemia Mielóide Crônica atípica BCR/ABL negativa, para fins terapêuticos.
Palavras-chave: Neoplasias mieloproliferativas, Leucemia mielóide crônica,transplante de medula óssea.
Introduction: Myeloproliferative diseases are neoplasms that originate from to tipotent stem cell, and is characterized by rapid proliferation of one or more bloodseries without significant dysplasia. They are classified into several subtypes including: Chronic Myeloid Leukemia, Chronic Neutrophilic Leukemia,Polycythemia Vera, Primary Mielofribrose and Unclassifiable chronic myeloproliferative syndrome. This classification is justified by genetic mutations that define the evolution and pathophysiology of each disease. Case report: We present a case of a female, 58, with nonspecific complaints, which in laboratoryfinding was suspected leukemia. Underwent clinical, laboratory, radiological,underwent bone marrow biopsy, was diagnosed with Unclassifiable chronic myeloproliferative syndrome.She was oriented to make use of hydroxyurea forerythrocyte control, but without improvement, started cytarabine. The patient lies onthe blast phase of the disease. Due to lack of remission of the disease, the patient wasreferred to the transplant list. Discussion: the case was properly conducted as to the diagnostic methods used in the investigation, the RT-PCR was fundamental for identification of Philadelphia chromosome. The medications used in the treatmentwas similar to the literature, however some doses, according to the survey were notthe same. The patient was refered to transplant, because there was no therapeutic response. Conclusion: This case report is a Nondescript Chronicles Myeloproliferative Syndrome, since it does not satisfy the standard diagnostic recommended by the World Health Organization, as it was classified as Atypical Chronic Myeloid Leukemia BCR / ABL negative for purposes therapeutic.
Keywords: myeloproliferative neoplasms, chronic leukemia, myeloid bone marrowtransplantation.
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