title: Pulmonary hypertension risk in patients with hemoglobin h disease: low incidence and absence of correlation with splenectomy.
abstract: Pulmonary hypertension (PHT) is a common complication for patients with β thalassemia intermediate (TI), especially splenectomized patients. However, the frequency and risk factors of PHT in patients with hemoglobin H (HbH) disease is unknown. The purpose of this study was to identify the prevalence of PHT risk manifested as tricuspid regurgitant jet velocity (TRV) ≥2.5 m/s in patients with HbH disease and its correlation with splenectomy. One hundred and ninety-eight patients with HbH disease who visited the 303rd Hospital of the People's Liberation Army (Nanning, China) were investigated. Thirteen subjects (6.5%) were diagnosed as having a risk of PHT. Regression analyses showed that the prevalence of PHT risk was correlated only with age (r = 0.195, p = 0.006) and not with splenectomy. The risk of PHT in patients older than 35 years was 5.7 times (range 1.8-18.6) greater than that for patients younger than 35 years. For splenectomized patients compared to those with HbH disease, patients with TI had a higher frequency of PHT risk, higher nucleated red blood cell counts (46.03 ± 41.11 × 10(9)/l vs. 0.18 ± 1.19 × 10(9)/l, p < 0.001) and a higher platelet counts (837.6 ± 178.9 × 10(9)/l vs. 506.7 ± 146.2 × 10(9)/l, p < 0.001). PHT risk is low in patients with HbH disease and does not correlate with splenectomy. Patients older than 35 years should be monitored regularly.
authors.affiliation: Department of Hematology, 303rd Hospital of the People's Liberation Army, Nanning, PR China.
authors.forename: Xiao-Lin
authors.initials: XL
authors.lastname: Yin
authors.forename: Xin-Hua
authors.initials: XH
authors.lastname: Zhang
authors.forename: Zhi-Kui
authors.initials: ZK
authors.lastname: Wu
authors.forename: Dong-Hai
authors.initials: DH
authors.lastname: Zhao
authors.forename: Ya-Li
authors.initials: YL
authors.lastname: Zhou
authors.forename: Yue-Hua
authors.initials: YH
authors.lastname: Yu
authors.forename: Tie-Niu
authors.initials: TN
authors.lastname: Liu
authors.forename: Su-Ping
authors.initials: SP
authors.lastname: Fang
authors.forename: Tian-Hong
authors.initials: TH
authors.lastname: Zhou
authors.forename: Li
authors.initials: L
authors.lastname: Wang
authors.forename: Jie
authors.initials: J
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D013156:Splenectomy; D017085:alpha-Thalassemia / Q000150:complications / Q000503:physiopathology / Q000601:surgery