Variable increases in
chitotriosidase levels have been reported in Italian patients with
beta-thalassemia major and intermedia. We measured plasma
chitotriosidase levels in Israeli patients with
beta-thalassemia to ascertain its use as a universal marker of disease and/or response to
therapy.
Chitotriosidase levels in 39 adults (16-53 years; 30 with
beta-thalassemia major, 9 with intermedia), and in 14 children (0.7-15 years; 12 with
beta-thalassemia major, 2 with intermedia) were compared with other measures of disease, such as
ferritin,
hemoglobin, liver function tests, and genotype. Plasma
chitotriosidase levels were normal (0.37 +/- 0.04 mU/mL) in all children. Twelve adults (31%) had elevated levels (>1.33 mU/mL): 11 patients (37%) with
thalassemia major and 1 patient (11%) with
thalassemia intermedia. A significant correlation was only found between plasma
chitotriosidase levels and
ferritin levels, and with mean number of transfusions per year. The patient with the highest
chitotriosidase (1,440 nmol/mL/hr) had the highest
ferritin (5,175 microg/L), required the most transfusions per year (40), and had abnormal liver tests. Normal
chitotriosidase levels in the pediatric cohort and increased levels in only some adults may reflect status of
iron overload in macrophages; 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, a more universal correlation was noted and
chitotriosidase levels were much higher.