Acute Panmyelosis with Myelofibrosis: A Rare Cause of Pancytopenia

Cláudia Falé, Ana Araújo, Sílvia Lourenço, Maria Pais


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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