Pulmonary hypertension in patients with chronic myeloproliferative disorders

Abstract

Pulmonary hypertension (PH) is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs) associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmonary hypertension in the context of CMPD's: chronic thromboembolic pulmonary hypertension, precapillary pulmonary hypertension and drug-induced PH. @ERSpublications Epidemiology, pathogenic mechanism and treatment approach of PH in patients with chronic myeloproliferative diseases http://ow.ly/Q1QX4

Pulmonary arterial hypertension exacerbated by ruxolitinib

“…The etiology of PH in myelofibrosis is complex, 15 but includes mechanisms that augment venous thrombosis and mechanisms resulting in PAH-like disease, such as extramedullary hemopoiesis. While these may respond differently to ruxolitinib, our patient had no evidence of pulmonary embolism, and ruxolitinib reduces extramedullary hematopoesis.…”

Complete resolution of idiopathic pulmonary arterial hypertension following chemotherapy

“…PH may occur due to tumour-related thrombotic or nonthrombotic pulmonary embolism (group IV), or in the presence of chronic myeloproliferative disorders or tumoural obstruction of pulmonary arteries (group V) [4]. Chronic myeloproliferative disorders are a heterogeneous group of diseases associated with the development of pre-capillary PH mimicking PAH [5]. Several reported cases revealed various pathogenic mechanisms implicated in the development of PH, such as drug-or stem cell transplantation-related PVOD, portal hypertension, splenectomy, extramedullary haematopoiesis or pulmonary leukaemic infiltration [5].…”

Reperfusion after pulmonary embolism - long-term follow-up, risk factors, clinical impact

“…It is however a well known fact that myeloproliferative diseases including polycythemia are predictors of both arterial and venous thrombosis as well as of CTEPH development following a PE episode 15,16 . The reason for thrombophilia in polycythemia patients is a greater blood viscosity leading to a greater arterial wall shear stress combined with formation of aggregates of red blood cells in arterioles and capillaries, which in turn leads to activation of platelets, prothrombogenic and pro-inflammatory state and, therefore, to disorders in resolution of thrombi 16 . Neither of these factors are however specific for primary polycythemia and can be associated with any increase in haemoglobin levels, which could be the explanation of our finding.…”