[Inherited primitive and secondary polycythemia]

Abstract

Myeloproliferative disorders and secondary polycythemia cover most of the polycythemia cases encountered in daily practice. Inherited polycythemias are rare entities that have to be suspected when the classical causes of acquired polycythemia have been ruled out. Recent advances were made in the understanding of these pathologies, which are still little known to the physicians. This review reports the state of knowledge and proposes an algorithm to follow when confronted to a possible case of inherited polycythemia.

Keywords: Chuvash polycythemia; Erythrocytosis; Hyperaffine hemoglobin; Hyperviscosity syndrome; Hémoglobine hyperaffine; Inherited polycythemia; Methemoglobinemia; Méthémoglobinémie; Polycythémie; Polyglobulie de Chuvash; Polyglobulie héréditaire; Syndrome d’hyperviscosité; Érythrocytose.