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   Am J Hematol 1993 Nov;44(3):219-20
   
Blue nasal secretions: a presentation of Gaucher's disease and concurrent
hemosiderosis.

    Schiano TD, Grinberg M, Nawabi I, Grabowski G
    
   Publication Types:
     * Letter
       
   PMID: 8213785, UI: 94027017
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   Blood Cells Mol Dis 1999 Feb;25(1):1-8
   
Plasma chitotriosidase activity in patients with beta-thalassemia.

    Barone R, Di Gregorio F, Romeo MA, Schiliro G, Pavone L
    
   Division of Pediatric Neurology, Institute of Pediatrics,
   Catania-Italy. r.barone@tau.it
   
   Chitotriosidase, a macrophage marker, which is extremely increased in
   plasma of Gaucher patients, was measured in patients with
   beta-thalassemia, an haematological disorder characterized by the
   genetic defect of beta-globin chains synthesis resulting in
   unproductive erythropoiesis and enormous expansion of the
   reticuloendothelial system. Plasma chitotriosidase was increased to a
   variable extent in 13 of 70 patients with beta-thalassemia major
   treated with the intense transfusion regimen and iron chelation
   therapy. It was normal in 22 and slightly elevated in 3 subjects with
   beta-thalassemia intermedia which were not transfused. The highest
   levels of plasma chitotriosidase, as high as in Gaucher patients, were
   found in 7 (10%) of the beta-thalassemia major patients which also had
   the highest degree of iron overload as judged by their serum ferritin
   level (> 3000 ng/ml), high SGPT level and elevated urinary iron
   excretion. To our knowledge, beta-thalassemia is hitherto the only
   disorder in which an increase of plasma chitotriosidase, comparable to
   that seen in Gaucher disease, may occur. The increase of plasma
   chitotriosidase activity in beta-thalassemia patients with high iron
   overload, could be related to an iron mediated damage to the lysosomal
   apparatus. In addition, similarly to Gaucher disease, the increased
   chitotriosidase production in beta-thalassemia might reflect
   macrophage activation probably related to the intracellular iron
   overload, storage of erythrocytes membrane break-down products and
   oxidation of excess alpha-hemoglobin subunits. Further studies are
   required to define the role of chitotriosidase evaluation to assess
   the efficacy of chelation therapy in reducing the macrophage
   activation due to intracellular iron overload in beta-thalassemia.
   
   PMID: 10349508, UI: 99279125
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