Table 1.

Proposed mechanism of pulmonary hypertension in myeloproliferative neoplasms.

Thrombocytosis with increased platelet activation and secretion of  PDGF or serotonin

Chronic thrombo-embolic pulmonary hypertension (CTEPH) □ Thrombotic occlusion of pulmonary vasculature □ Splenectomy

Portal hypertension

High output states

Drug induced □ Pulmonary veno-occlusive disease (anagrelide) □ Pulmonary arterial hypertension (dasatinib and interferon alpha/beta)

Aberrant JAK/Src-STAT3 signaling pathway □ Promotes inflammation □ Promotes abnormal hyper-proliferative state □ Enhances abnormal angiogenesis □ Depletes endothelial nitric oxide stores

Pulmonary vascular remodeling by myeloid cells

PDGF, platelet-derived growth factor; JAK, Janus Kinase; STAT, signal transducer and activator of transcription.