Acute Panmyelosis with Myelofibrosis: A Rare Cause of Pancytopenia

Authors

  • Cláudia Falé Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.
  • Ana Araújo Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.
  • Sílvia Lourenço Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.
  • Maria Pais Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.

DOI:

https://doi.org/10.20344/amp.315

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.

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Author Biographies

Cláudia Falé, Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.

Ana Araújo, Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.

Sílvia Lourenço, Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.

Maria Pais, Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.

Published

2013-10-31

How to Cite

1.
Falé C, Araújo A, Lourenço S, Pais M. Acute Panmyelosis with Myelofibrosis: A Rare Cause of Pancytopenia. Acta Med Port [Internet]. 2013 Oct. 31 [cited 2024 Mar. 28];26(5):613-6. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/315