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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