Cooley’s anemia treatment

Concerted electron transfer oxidases, 683 Control processes biology calcium, 591 Cooley s anemia treatment... 

In the treatment of poisoning by lead or other metal ions, higher concentrations of chelant can be safely obtained in humans by administering Na2CaEDTA rather than Na EDTA. The metal ion is bound by displacing small amounts of Ca " that the body can tolerate. Use of Na EDTA would result in calcium chelation and thus serious depletion of calcium in the body fluids (44). Removal of iron in Cooley s anemia is accompHshed by using chelants that are relatively specific for iron (45). 

Anticancer, antiviral, treatment of arthritis, antibacterial, antifungal, antifouling, treatment of Cooley s anemia, algicides, molluscicides, contrast agents, radiology agents... 

Characteristics of the more common /3-thalassemia syndromes are summarized in Table 28-4. Not included are two different abnormal heterozygotes. Anemia results from precipitation of excess a-globin chains, premature red cell destruction in bone marrow and the circulation, and deficiency of functional hemoglobin tetramer. /S-Thalassemia major, or Cooley s anemia, occurs when /3-globin synthesis is markedly depressed or absent. The ineffective erythropoiesis causes massive erythroid proliferation, skeletal deformities, and extramedullary erythropoiesis. The usual treatment is frequent blood transfusion. 

Derivatives Bickel etal., ibtd. 46, 1385 (1963). Clinical effi-cacy in thallasemia major (Cooley s anemia) B. Modell et al, Brit. MedL J. 284, 1081 (1982). Treatment of hemodialysis-induced aluminum accumulation in brain P. Ackril el al. Lancet 2, 692 (1980) in boue H. H. Malluche et al, N. Engl J. Med. 311, 140(1984). Neurotoxicity study N. F. Olivieri et aL ibid. 314, 869 (1986). Review of clinical ex-... 

Certain types of anemia require regular transfusions of whole bloodi since the victims of these diseases cannot correctly manufacture their own. One such disorder, -thalassemla. Is described In more detail elsewhere In this publication. Because the body lacks any mechanism for excreting significant amounts of serum Iron, the Iron contained In transfused blood ( 250 mg per pint) can accumulate to lethal levels. At this time the only treatment for Cooley s anemia Is continual transfusion and so some way must be found to efficiently remove this excess Iron. Thus there Is an obvious need for a drug i lch can selectively bind Iron in vivo and facilitate Its excretion. 

Anderson, W. F. "Iron Chelation In the Treatment of Cooley s Anemia" this publication. 

Cooley s anemia (P-thalassemia major), with chronic transfusional iron overload, probably best illustrates the hurdles which must be overcome in chelator design. The lifelong treatment necessary in this disorder dictates that both the long-term toxicity of the drug and the efficiency wiA which the ligand... 

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