Diagnostic, evolutionary and therapeutic aspects of primary myelofibrosis

Revista Cubana de Hematología, Inmunología y Hemoterapia

Espinosa Estrada, Edgardo¹  Izquierdo Cano, Lissete¹  Ávila Cabrera, Onel¹  Rodríguez, Luis Ramón¹  González Albelo, Yanet¹  Bencomo Hernández, Antonio¹  Espinosa Martínez, Edgardo¹ 

[1] Instituto de Hematología e Inmunología, La Habana, Cuba

关键词: Primary myelofibrosis;    survival;   

DOI  :  

学科分类：生理学与病理学

来源: SciELO

【 摘 要 】

Primary myelofibrosis is characterized by clonal expansion of hematopoietic stem cell with a non-reactive clonal proliferation of fibroblasts and bone marrow fibrosis, which occurs at an extramedullary hematopoiesis. Since it is a rare disease for which no research has been conducted in our environment, we charcterized the diagnostic aspects, treatment and evolution in patients treated at the Hematology and Immunology Institute during the period between November 1992 and May 2009. The most frequent presentation was in the group older than 60 years. 80 % of patients had symptoms at diagnosis. The most frequent were the left upper quadrant discomfort, fever, night sweats and weight loss. The main findings in the blood count at this disease onset were anemia and thrombocytosis followed by high leukocyte counts with leukoerythroblastic reaction in 80 % of cases. There were high levels of LDH 78.6 %. The bone marrow biopsy was 60 % pre fibrotic stage. Patients who died were in the highest risk groups. The median survival was approximately 5.5 years.

【 授权许可】

CC BY-NC