Hypertension artérielle pulmonaire et hémopathies malignes

Abstract

Many recent publications have evidenced a statistical link between pulmonary arterial hypertension and myeloproliferative disorders, once excluded secondary causes of pulmonary arterial hypertension (more particularly, portal hypertension or thromboembolic events). Nonetheless, the frequency of such an association is quite variable, from 10 to 50 % and depends both on the population studied and on the type of myeloproliferative disorder. The most robust association is observed with essential thrombocythemia. Pulmonary arterial hypertension has also been described in association with lymphoma, acute leukemia, myeloma or large granular lymphocyte proliferation. The absence of large series call for a prospective study of pulmonary arterial hypertension in malignant hematologic disorders to firmly establish the exact frequency of such an association, to define more precisely its physiopathology in order to elaborate more specific diagnostic criteria together with guidelines for both treatment and follow-up.