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Sarcoma mielóide isolado com atingimento multiorgânico : um caso de sucesso : caso clínico e revisão da literatura
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O Sarcoma Mielóide (SM) é um tumor extramedular que geralmente ocorre em associação com outras doenças hematológicas, como leucemia mieloide aguda (LMA), síndromes mielodisplásicos e neoplasias mieloproliferativas crónicas, correspondendo a uma proliferação de células mielóides imaturas. Os casos em que o SM é a primeira manifestação da doença, sem história ou evidência de leucemia mielóide, são raros e geralmente evoluem para LMA.
Apresentamos o caso de uma doente de 25 anos com queixas de dor sacro-ilíaca e perda ponderal durante 7 meses, altura em que a biópsia de lesões cutâneas, permitiu o diagnóstico de SM isolado. A avaliação subsequente demonstrou a presença de lesões mieloblásticas pancreáticas, renais e ósseas, assim como infiltração do SNC.
A doente foi tratada com quimioterapia intratecal e dois ciclos de quimioterapia sistémica, com regressão de todas as lesões neoplásicas, seguidos de transplante alogénico de células hematopoiéticas. Durante todo o processo não houve qualquer evidência de proliferação blástica na medula óssea ou no sangue periférico.
A doente encontra-se atualmente em follow-up e livre de doença há 80 meses.
O SM é uma entidade frequentemente mal ou tardiamente diagnosticada, como ocorreu no caso clínico presente.
Apesar de os resultados terapêuticos serem maus, estudos recentes mostram que o SM Isolado tem melhor prognóstico que a LMA.
Myeloid sarcoma (MS) is a rare extramedullary tumor of immature myeloid cells. It usually occurs with other hematologic disorders such as acute myeloid leukemia (AML), myelodysplastic syndrome or chronic myeloproliferative neoplasms. The cases presenting as isolated myeloid sarcomas, without history or evidence of myeloid leukemia, are rare and typically progress to form AML. We present a case of a 25-year-old female patient who experienced sacroiliac pain and weight loss for 7 months, before being diagnosed with isolated MS. A skin lesion biopsy revealed infiltration by myeloblastic cells. The following work-up revealed the presence of pancreatic, renal and bone myeloblastic lesions as well as CNS infiltration. The patient was treated with intrathecal chemotherapy and two cycles of systemic chemotherapy, with regression of all neoplastic lesions, followed by allogenic hematopoietic cell transplant. During the entire process, there was no evidence of blastic proliferation either in the blood or the bone marrow. The patient is currently still on follow-up and free of disease for 80 months. Myeloid Sarcoma is commonly late or misdiagnosed, as it was in the present case. Despite having generally poor outcomes, recent studies reveal that Isolated MS has a better prognosis than AML.
Myeloid sarcoma (MS) is a rare extramedullary tumor of immature myeloid cells. It usually occurs with other hematologic disorders such as acute myeloid leukemia (AML), myelodysplastic syndrome or chronic myeloproliferative neoplasms. The cases presenting as isolated myeloid sarcomas, without history or evidence of myeloid leukemia, are rare and typically progress to form AML. We present a case of a 25-year-old female patient who experienced sacroiliac pain and weight loss for 7 months, before being diagnosed with isolated MS. A skin lesion biopsy revealed infiltration by myeloblastic cells. The following work-up revealed the presence of pancreatic, renal and bone myeloblastic lesions as well as CNS infiltration. The patient was treated with intrathecal chemotherapy and two cycles of systemic chemotherapy, with regression of all neoplastic lesions, followed by allogenic hematopoietic cell transplant. During the entire process, there was no evidence of blastic proliferation either in the blood or the bone marrow. The patient is currently still on follow-up and free of disease for 80 months. Myeloid Sarcoma is commonly late or misdiagnosed, as it was in the present case. Despite having generally poor outcomes, recent studies reveal that Isolated MS has a better prognosis than AML.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2016
Palavras-chave
Sarcoma mielóide Hematologia