Sociedad Argentina de Hematología

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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Revista Argentina de Hematología

Abstract

Volumen:    21    # Number : XXIII Congreso Argentino de Hematología

Publication Date :    NOVIEMBRE    Year:    2017

   POLIGLOBULIAS NO CLONALES

Non clonal erythrocytosis

Authors: Chiappe G

Abstract: Erythrocytosis is always a pathological event, more frequently a salvage mechanism for preventing an inadequate oxygen delivery to tissues than a deleterious condition. Both situations must be properly differentiated in order to afford the most appropriate therapeutic approach with or without phlebotomies. Erythrocytosis is classified as primary, when the defect lies in the bone marrow erythroid precursor, and secondary, when there is an increase in the renal or extrarenal erythropoietin production. In a group of secondary eythrocytosis caused by mutations in hypoxia sensing pathway related genes, erythrocytosis is only one of a handful of consequences from the increased hypoxia inducible factor activity. In some circumstances secondary erythocytosis determines an excessive increment in blood viscosity, with the consequent reduction in blood flow and oxygen tissue supply. In these cases phlebotomies must also be indicated in order to break this vicious circle. The first entity to be discarded is polycythemia vera through clinical and hematological picture and JAK2 mutations, then the large list of secondary (acquired or hereditary, as hemoglobinopathies) etiologies through a thoughtful investigation, and finally, if no pathogenic cause has yet been disclosed, the disregulation of the hypoxemic response pathway must be considered and, if possible, properly evaluated. A positive family history may indicate an autosomic dominant inheritance (not infrequent in this pathology) but also the effect of an environmental factor on different family members.

Key words: erythrocytosis, hipoxia, erythropoietin, blood viscosity.

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SOCIEDAD ARGENTINA DE HEMATOLOGÍA
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