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  • Title: [Acute panmyelosis with myelofibrosis: a rare cause of pancytopenia].
    Author: Falé C, Araújo A, Lourenço S, Pais M.
    Journal: Acta Med Port; 2013; 26(5):613-6. PubMed ID: 24192103.
    Abstract:
    Acute Panmyelosis with myelofibrosis is a rare and aggressive acute myeloid leukemia accounting for 2% of all acute leukemias. Clinically, it is characterized by an acute onset of cytopenias associated with extensive bone marrow fibrosis in the absence of splenomegaly and related morphological changes in the red blood cells such as dacryocytes. The presence of fibrosis further complicates the correct diagnosis of this rare disease, being important to differentiate this entity from other hematologic malignancies such as other acute myeloid leukemias and myelodysplastic syndromes associated with myelofibrosis. We report a case of a young adult of 16 years old, admitted with severe pancytopenia. The exposure of this case is justified by the rarity of this pathology associated with few cases reported in medical literature, allowing for a clinical sharing and a better understanding of the different clinical aspects of this entity of difficult diagnosis. Panmielose aguda com mielofibrose é uma forma rara e agressiva de Leucemia mielóide aguda que corresponde a 2% de todas as leucemias agudas. Clinicamente caracteriza-se por um início abrupto de citopenias, associadas a extensa fibrose da medula óssea e uma proliferação de percussores das três séries mieloides (panmielose). O seu rápido curso, ao contrário de outras hemopatias de carácter insidioso não se acompanha de esplenomegalia sugestiva de hematopoese extramedular ou alterações dismórficas da série rubra com o aparecimento de dacriocitos. O desafio diagnóstico na panmielose aguda com mielofibrose resulta do diagnóstico diferencial quer com síndromes mielodisplasicos associados a mielofibrose quer com outras leucemias agudas. Reportamos o caso de um jovem de 16 anos, internado por pancitopenia, cuja marcha diagnostica, conduziu ao diagnóstico de panmielose aguda com mielofibrose. A exposição deste caso justifica-se pela raridade desta patologia associada aos escassos casos descritos na literatura, permitindo uma partilha clinica e melhor compreensão desta entidade de difícil diagnóstico.
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