Pulmonary hypertension in patients with Philadelphia-negative myeloproliferative neoplasms: a single-center retrospective analysis of 225 patients

Abstract

Background The prevalence of pulmonary hypertension (PH) in myeloproliferative neoplasms (MPNs) varies among studies. We analyzed the prevalence of PH in Korean patients with Philadelphia-negative (Ph-) MPNs. Methods Medical records of patients with Ph-MPNs [essential thrombocythemia (ET), polycythemia vera (PV), or primary myelofibrosis (PMF)] visiting a single hospital between 1993 and 2019 were reviewed retrospectively. Transthoracic echocardiographic examination (TTE) results were reviewed and PH was diagnosed according to established guidelines.

Pulmonary hypertension in patients with chronic myeloid leukemia

“…[7] Many reports have described PH in patients with Philadelphia chromosome-negative MPN (Ph À MPN). [8][9][10][11][12][13] By contrast, there have been few reports on PH in newly diagnosed CML patients. Dasatinib, a dual Src and BCR-ABL TKI, is associated with PAH in patients with CML.…”

Myeloproliferative neoplasm-driving Calr frameshift promotes the development of pulmonary hypertension in mice

“…Fukushima Medical University, Fukushima, Japan. 6 Department of Diagnostic Pathology, School of Medicine, Fukushima Medical University, Fukushima, Japan.…”

“…Bone marrow (BM)-derived cells and perivascular inflammatory infiltrates contribute to PA remodeling in PH [ 2 , 3 ]. Among 5 etiological groups, the WHO group-V PH encompasses multifactorial mechanisms, including MPNs, which are often complicated by PH, with 5%-60% of the prevalence [ 4 – 6 ]. MPN-related PH is associated with crucial features, such as thromboembolism and hypermetabolic state [ 5 ].…”

“…To date, most of studies for PH in MPN patients lack information about driver mutations, although a retrospective study indicated higher prevalence of CALR mutations in ET patients with PH than those without [ 6 ]. Besides megakaryocyte lineage with TpoR expression, a recent study indicated that transcriptional misregulation occurs with JAK-STAT activation in CALR -mutated PB-MNCs similar to JAK2 -mutated PB-MNCs [ 12 ].…”

“…To date, most of studies for PH in MPN patients lack information about driver mutations, although a retrospective study indicated higher prevalence of CALR mutations in ET patients with PH than those without [6]. Red, blue, and orange dots represent cells from Calr WT/WT mice, Calr del10/WT mice, and negative control (mixture of WT and Calr del10 cells), respectively.…”