Sociedad Argentina de Hematología

Revista Hematología

 

 

 

 

 

Revista Argentina de Hematología

Abstract

Volumen:    17    # Number : 3

Publication Date :    Septiembre - Diciembre    Year:    2013

   ARTÍCULO DE REVISIÓN

Some coments about Primary Myelofibrosis asociated Anemia

Authors: Lanari Zubiaur EA, Romero Maciel MA, Erro MG, Martin AE

Abstract: Primary Myelofibrosis (MFP) is one of the Myeloproliferative Neoplasms with worst prognosis and is associated with low survival and a high probability of leukemic transformation. JAK2v617F mutation is seen in about 60 % of cases and is responsible, by different mechanisms, for many of the clinical manifestations associated with this pathology. The anemia, with a multifactorial origin, evidenced by up to 50 % during the course of the disease becomes an increasingly important role not only in the quality of life, but also in the patient’s prognosis. There are multiples current treatments to combat it. Turn out to be the most effective: RBC transfusion, EPO mimetics, Androgens and Immunomodulators. Special mention deserves the anemia due to EPO receptor inhibition by JAK Inhibitor, Ruxolitinib. It has an incidence of 30-40 % and is observed from the fourth week, tending to a gradual recovery of hemoglobin levels that tend to gradually stabilize from week 24. Their management is primarily based on transfusions and dose reduction, although other options as EPO/ Androgens are useful. This work aims to address the problem of anemia associated with MFP due to the increasingly known impact on prognosis in this disease, emphasizing on the different treatment options tailored to the individual characteristics of each patient.

Key words: Myelofibrosis, Anemia Treatment, Ruxolitinib.

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