Plasma chitotriosidase activity in patients with. beta-thalassemia

ABSTRACT

Variable increase in plasma chitotriosidase levels have been reported in Italian patients with beta thalassemia major and intermedia. We measured plasma chitotriosidase levels in Egyptian patients with beta thalassemia to ascertain its use as a universal marker of the disease and /or response to therapy. Chitotriosidase levels in 30 children [1.5-15 years] with beta thalassemia major and 10 children [6-15 years] with beta thalassemia intermedia were compared with other measures of the disease, such as ferritin, hemoglobin F, pre-transfusion hemoglobin, and liver function tests. Plasma chitotriosidase levels were normal in all cases with thalassemia intermedia [median 22.5, range 8.8-69 nmol/ml/h]. Eight patients with thalassemia major had elevated levels [<69.4 nmol/ml/h]. A significant correlation was found between plasma chitotriosidase and hemoglobin F, ferritin levels, and with duration of transfusion. Normal chitotriosidase levels in thalassemia intermedia and elevated levels only with thalassemia major and correlation with duration of blood transfusion may reflects status of iron overload in macrophages. The highly significant correlation with hemoglobin F might reflect the role of thalassemia genotype in chitotriosidase production. Thus there may be a role for monitoring chitotriosidase in patients with beta thalassemia. Our results confirm results of the Italian cohort; however in the latter chitotriosidase levels were much higher