Abstract

RODRIGUEZ FRAGA, Yusaima. Acute panmyelosis with myelofibrosis. Rev cubana med [online]. 2021, vol.60, n.1  Epub Feb 25, 2021. ISSN 1561-302X.

Acute panmyelosis with myelofibrosis (PMAF) is a rare hematologic disorder, defined as a subtype of acute leukemia. A 31-year-old male patient with a history of marked decay, evening fever, and gingivorrhagia is reported. The physical examination showed mucous skin paleness, slight splenomegaly and the complementary examinations showed pancytopenia with 5% blasts. In the medullogram no material was obtained and the imprint suggests non-promyelocytic acute myeloid leukemia. Immunophenotype study by flow cytometry confirmed an increase in myeloblasts positive for (CD34, CD13, CD17, CD117, CD38) and decreased in (CD11c and HLA-DR), with marked myelofibrosis in bone marrow biopsy without the presence of blasts. He received induction treatment with a 3 + 7 scheme (Citosar + Rubidomycin) after which the patient was in hematological remission with persistence of spinal fibrosis. Later, he started treatment with lenalidomide, bisphosphonates (Zoledronic Acid) and was in hematological remission 11 months after diagnosis, until March 2020. Histocompatibility studies (HLA) were performed for allogeneic transplantation.

Keywords : acute panmyelosis with myelofibrosis; AML; myeloproliferative diseases; myelofibrosis.

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