Blood Research

Characteristics and clinical features of MPN patients who had pulmonary hypertension at the time of diagnosis.

No. Age (yr)/gender Diagnosis Prognostication Driver gene mutations WBC (×109/L) Hb (g/dL) Platelet (×109/L) LDH (×UNL) Thrombotic eventsa) Comorbidities TRV (m/sec) RVSP (mmHg) Late thrombotic eventsb) Follow-up duration (yr) Alive/dead
1 75/F ET R-IPSET-T high JAK2V617F 16.5 11.1 2,299 1.2 None None 3.0 41 No 4.3 Alive
2 79/F ET R-IPSET-T high JAK2V617F 10.5 14.2 896 1.0 CI, IHD HT, DL 3.7 60 No 1.2 Alive
3 80/F PV - JAK2V617F 20.7 16.3 492 1.3 DVT DM, HT 3.4 51 No 1.0 Alive
4 56/M PMF IPSS low JAK2V617F 17.7 14.7 1,273 1.0 None CKD 3.0 41 No 4.0 Alive
5 69/M PMF IPSS high JAK2V617F 31.1 11.0 570 1.6 None CKD 3.0 41 No 2.4 Alive
6 71/F PMF IPSS high JAK2V617F 51.2 10.2 786 4.7 None DM 4.1 72 No 4.1 Alive
7 79/M PMF IPSS intermediate-2 CALR 9.3 9.2 579 1.5 None CKD 2.9 40 No 2.4 Alive
8 82/F PMF IPSS intermediate-1 JAK2V617F 19.7 12.2 2,178 1.6 None None 3.1 43 No 0.6 Alive

a)Thrombotic events prior to or at the time of diagnosis. b)Thrombotic events after diagnosis.

Abbreviations: CALR, calreticulin; CI, cerebral infarction; CKD, chronic kidney disease; DL, dyslipidemia; DM, diabetes mellitus; DVT, deep vein thrombosis; ET, essential thrombocythemia; F, female; HT, hypertension; IHD, ischemic heart disease; IPSS, International Prognostic Scoring System; LDH, lactate dehydrogenase; M, male; MPN, myeloproliferative neoplasm; PMF, primary myelofibrosis; PV, polycythemia vera; R-IPSET-T, revised International Prognostic Score for Essential Thrombocythemia-thrombosis; RVSP, right ventricle systolic pressure; TRV, tricuspid regurgitation velocity; UNL, upper normal limit.

Blood Res 2020;55:77~84 https://doi.org/10.5045/br.2020.2020001
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