Thalassemia Cure
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Is this a .. cure .. ?
Iron depletion BEFORE transfusions.
Alternative treatment paradigm for thalassemia using iron chelators.
Szuber N, Buss JL, Soe-Lin S, Felfly H, Trudel M, Ponka P
Exp Hematol 2008 May 2.
OBJECTIVE:
beta-thalassemia major, or Cooley's anemia, is a red blood cell
disorder requiring lifelong blood transfusions for survival.
Erythrocytes accumulate toxic iron at their membranes, triggering an
oxidative cascade that leads to their premature destruction in high
numbers. We hypothesized that removing this proximate iron compartment
as a primary treatment, using standard and alternative orally active
iron chelators, could prevent hastened red cell removal and,
clinically, perhaps alleviate the need for transfusion.
MATERIALS AND METHODS:
Iron chelators of the pyridoxal isonicotinoyl hydrazone family
(pyridoxal isonicotinoyl hydrazone and its analog pyridoxal ortho-
chlorobenzoyl hydrazone) were evaluated in addition to the present
mainstay, desferrioxamine and deferiprone, in vitro and in vivo.
RESULTS:
Treatment of human beta-thalassemic erythrocytes with chelators
resulted in significant depletion of membrane-associated iron and
reduction of oxidative stress, as evaluated by methemoglobin levels.
When administered to beta-thalassemic mice, iron chelators mobilized
erythrocyte membrane iron, reduced cellular oxidation, and prolonged
erythrocyte half-life. The treated thalassemic mice also showed
improved hematological abnormalities. Remarkably, a beneficial effect
as early as the erythroid precursor stage was manifested by normalized
proportions of mature vs immature reticulocytes. All four compounds
were also found to mitigate iron accumulation in target organs, a
critical determinant for patient survival. In this respect, pyridoxal
ortho-chlorobenzoyl hydrazone displayed higher activity relative to
other chelators tested, further diminishing iron in liver and spleen
by up to approximately fivefold and twofold, respectively.
CONCLUSION:
Our study demonstrates the ability of iron chelators to improve
several of the fundamental pathological disturbances of thalassemia,
and reveals their potential for clinical use in diminishing
requirement for transfusion when administered early in disease
development.
Experimental hematology [Exp Hematol]
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