The need of splenectomy in thalassemia major is more likely where the disease is not suppressed efficiently by transfusion treatment. The aim of this report has been to evaluate the proportion of patients for whom splenectomy has been avoided or delayed in a large cohort of thalassemic patients during a 40-year span. A series of 872 regularly transfused beta thalassemia patients born between 1960 and 1999 was pooled from the records of WebThal, a thalassemia dedicated software in five Italian Centers. For each patient's date of birth, first transfusion and splenectomy were considered. Kaplan-Meier and Wilcoxon tests for group comparison were applied. Age at splenectomy and date of splenectomy correlated positively (r = 0.73, P < 0.001). The probability to undergo surgery within the first 10 years of life was 57, 22, 6, and 7%, respectively, for patients born in the 1960s, 1970s, 1980s, and 1990s. For thalassemic patients on standard treatment, the chance to be splenectomized is today low during childhood and young adulthood. Further studies are needed to quantify the specific contribution of the presence of the spleen to the prolonged survival and quality of life in well-treated thalassemic patients.

Changing patterns of splenectomy in transfusion-dependent thalassemia patients / A. Piga, M. Serra, F. Longo, G. Forni, G. Quarta, M. D. Cappellini, R. Galanello. - In: AMERICAN JOURNAL OF HEMATOLOGY. - ISSN 0361-8609. - 86:9(2011 Sep), pp. 808-810. [10.1002/ajh.22102]

Changing patterns of splenectomy in transfusion-dependent thalassemia patients

M.D. Cappellini
Penultimo
;
2011

Abstract

The need of splenectomy in thalassemia major is more likely where the disease is not suppressed efficiently by transfusion treatment. The aim of this report has been to evaluate the proportion of patients for whom splenectomy has been avoided or delayed in a large cohort of thalassemic patients during a 40-year span. A series of 872 regularly transfused beta thalassemia patients born between 1960 and 1999 was pooled from the records of WebThal, a thalassemia dedicated software in five Italian Centers. For each patient's date of birth, first transfusion and splenectomy were considered. Kaplan-Meier and Wilcoxon tests for group comparison were applied. Age at splenectomy and date of splenectomy correlated positively (r = 0.73, P < 0.001). The probability to undergo surgery within the first 10 years of life was 57, 22, 6, and 7%, respectively, for patients born in the 1960s, 1970s, 1980s, and 1990s. For thalassemic patients on standard treatment, the chance to be splenectomized is today low during childhood and young adulthood. Further studies are needed to quantify the specific contribution of the presence of the spleen to the prolonged survival and quality of life in well-treated thalassemic patients.
pulmonary-hypertension; beta-thalassemia; complications; survival
Settore MED/09 - Medicina Interna
set-2011
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/162028
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