BACKGROUND: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). METHODS: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. RESULTS: The mean age of Group I patients at development of PHT was 37.3±10.6years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≥300×10(6)/l (2.59-times) or a previous history of thromboembolic events (3.69-times). CONCLUSION: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.
BACKGROUND:Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). METHODS: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. RESULTS: The mean age of Group I patients at development of PHT was 37.3±10.6years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≥300×10(6)/l (2.59-times) or a previous history of thromboembolic events (3.69-times). CONCLUSION: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.
Authors: Claudia R Morris; Hae-Young Kim; Elizabeth S Klings; John Wood; John B Porter; Felicia Trachtenberg; Nancy Sweeters; Nancy F Olivieri; Janet L Kwiatkowski; Lisa Virzi; Kathryn Hassell; Ali Taher; Ellis J Neufeld; Alexis A Thompson; Sandra Larkin; Jung H Suh; Elliott P Vichinsky; Frans A Kuypers Journal: Br J Haematol Date: 2015-04-24 Impact factor: 6.998
Authors: Claudia R Morris; Hae-Young Kim; John Wood; John B Porter; Elizabeth S Klings; Felicia L Trachtenberg; Nancy Sweeters; Nancy F Olivieri; Janet L Kwiatkowski; Lisa Virzi; Sylvia T Singer; Ali Taher; Ellis J Neufeld; Alexis A Thompson; Vandana Sachdev; Sandra Larkin; Jung H Suh; Frans A Kuypers; Elliott P Vichinsky Journal: Haematologica Date: 2013-04-12 Impact factor: 9.941
Authors: Sylvia T Singer; Frans Kuypers; Jeffery Fineman; Ginny Gildengorin; Sandra Larkin; Nancy Sweeters; Howard Rosenfeld; Gregory Kurio; Annie Higa; Michael Jeng; James Huang; Elliott P Vichinsky Journal: Ann Hematol Date: 2014-02-28 Impact factor: 3.673