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Continuous RBC Transfusions in a Patient With Sickle Cell Disease

Continuous RBC Transfusions in a Patient With Sickle Cell Disease Abstract A patient with severe sickle cell anemia received approximately 600 RBC transfusions during a period of 30 years. Continuous prophylactic transfusions were administered for 13 years. During the 13-year period, except for tissue damage already inflicted by sickle cell disease, she was free of symptoms. When a hemoglobin concentration of 12 g/dL was maintained, she was shown to have ineffective erythropoiesis postulated to be due to the inability of her reticulocytes to cross the sinusoidal barrier from marrow to blood. Up to the time of her death at the age of 47 years, there was no evidence that her massive iron overload produced clinical symptoms other than those related to osteoporosis. The cause of her death was unexplained, although her massive iron overload remains suspect. (Arch Intern Med 1982;142:279-282) References 1. Russell MO, Goldberg HI, Reis L, et al: Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. J Pediatr 1976;88:382-387.Crossref 2. Ricks P: Further experience with exchange transfusion in sickle cell anemia and pregnancy. Am J Obstet Gynecol 1968;100:1087-1091. 3. Wilimas J, Goff JR, Anderson HR, et al: Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents. J Pediatr 1980;96:205-208.Crossref 4. Sarnaik S, Soorya D, Kim J, et al: Periodic transfusions for sickle cell anemia and CNS infarction. Am J Dis Child 1979;133:1254-1257. 5. Brody JI, Goldsmith MH, Park SK, et al: Symptomatic crises of sickle cell anemia treated by limited exchange transfusion. Ann Intern Med 1970;72:327-330. 6. Finch CA, Deubelbeiss K, Cook JD, et al: Ferrokinetics in man. Medicine 1970;49:17-53.Crossref 7. Giblett ER, Coleman DH, Pirzio-Biroli G, et al: Erythrokinetics: Quantitative measurements of red cell production and destruction in normal subjects and patients with anemia. Blood 1956;11:291-309. 8. Finch CA, Hosain F, Morgan EH, et al: The ferrokinetic approach to anemia. Ser Haematol 1965;6:30-40. 9. Graham G, Bates GW, Rachmilewitz EA, et al: Nonspecific serum iron in thalassemia: Quantitation and chemical reactivity. Am J Hematol 1979;6:207-217.Crossref 10. Pootrakul P, Hungsprenges S, Baylink D, et al: Relationship between erythropoiesis and bone metabolism in thalassemia. N Engl J Med 1981;304:1470-1473.Crossref 11. Hillman RS, Finch CA: Erythropoiesis, normal and abnormal. Sem Hematol 1967;4:327-336. 12. Donegan CC, Macilwaine WA, Leavell BS: Hematologic studies on patients with sickle cell anemia following multiple transfusions. Am J Med 1954;17:29-35.Crossref 13. Chaplin H, Keitel HG, Peterson RE: Hematological observations on patients with sickle cell anemia sustained at normal hemoglobin levels by multiple transfusions. Blood 1956;11:834-845. 14. Tinsley JC, Moore CV, Dubach R, et al: The role of oxygen in the regulation of erythropoiesis: Depression of the rate of delivery of new RBCs to the blood by high concentrations of inspired oxygen. J Clin Invest 1949;28:1544-1564.Crossref 15. Reinhard EH, Moore CV, Dubach R, et al: Depressant effects of high concentrations of inspired oxygen on erythrocytogenesis: Observations on patients with sickle cell anemia with a description of the observed toxic manifestations of oxygen. J Clin Invest 1944;23:682-698.Crossref 16. Papayannopoulou T, Finch CA: Radioiron measurements of RBC maturation. Blood Cells 1975;1:535-546. 17. Labardini J, Papayannopoulou T, Cook JD, et al: Marrow radioiron kinetics. Haematologia 1973;7:301-312. 18. Wapnick AA, Lunch SR, Krawitz P, et al: Effects of iron overload on ascorbic acid metabolism. Br Med J 1968;3:704-707.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Continuous RBC Transfusions in a Patient With Sickle Cell Disease

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Publisher
American Medical Association
Copyright
Copyright © 1982 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1982.00340150079015
Publisher site
See Article on Publisher Site

Abstract

Abstract A patient with severe sickle cell anemia received approximately 600 RBC transfusions during a period of 30 years. Continuous prophylactic transfusions were administered for 13 years. During the 13-year period, except for tissue damage already inflicted by sickle cell disease, she was free of symptoms. When a hemoglobin concentration of 12 g/dL was maintained, she was shown to have ineffective erythropoiesis postulated to be due to the inability of her reticulocytes to cross the sinusoidal barrier from marrow to blood. Up to the time of her death at the age of 47 years, there was no evidence that her massive iron overload produced clinical symptoms other than those related to osteoporosis. The cause of her death was unexplained, although her massive iron overload remains suspect. (Arch Intern Med 1982;142:279-282) References 1. Russell MO, Goldberg HI, Reis L, et al: Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. J Pediatr 1976;88:382-387.Crossref 2. Ricks P: Further experience with exchange transfusion in sickle cell anemia and pregnancy. Am J Obstet Gynecol 1968;100:1087-1091. 3. Wilimas J, Goff JR, Anderson HR, et al: Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents. J Pediatr 1980;96:205-208.Crossref 4. Sarnaik S, Soorya D, Kim J, et al: Periodic transfusions for sickle cell anemia and CNS infarction. Am J Dis Child 1979;133:1254-1257. 5. Brody JI, Goldsmith MH, Park SK, et al: Symptomatic crises of sickle cell anemia treated by limited exchange transfusion. Ann Intern Med 1970;72:327-330. 6. Finch CA, Deubelbeiss K, Cook JD, et al: Ferrokinetics in man. Medicine 1970;49:17-53.Crossref 7. Giblett ER, Coleman DH, Pirzio-Biroli G, et al: Erythrokinetics: Quantitative measurements of red cell production and destruction in normal subjects and patients with anemia. Blood 1956;11:291-309. 8. Finch CA, Hosain F, Morgan EH, et al: The ferrokinetic approach to anemia. Ser Haematol 1965;6:30-40. 9. Graham G, Bates GW, Rachmilewitz EA, et al: Nonspecific serum iron in thalassemia: Quantitation and chemical reactivity. Am J Hematol 1979;6:207-217.Crossref 10. Pootrakul P, Hungsprenges S, Baylink D, et al: Relationship between erythropoiesis and bone metabolism in thalassemia. N Engl J Med 1981;304:1470-1473.Crossref 11. Hillman RS, Finch CA: Erythropoiesis, normal and abnormal. Sem Hematol 1967;4:327-336. 12. Donegan CC, Macilwaine WA, Leavell BS: Hematologic studies on patients with sickle cell anemia following multiple transfusions. Am J Med 1954;17:29-35.Crossref 13. Chaplin H, Keitel HG, Peterson RE: Hematological observations on patients with sickle cell anemia sustained at normal hemoglobin levels by multiple transfusions. Blood 1956;11:834-845. 14. Tinsley JC, Moore CV, Dubach R, et al: The role of oxygen in the regulation of erythropoiesis: Depression of the rate of delivery of new RBCs to the blood by high concentrations of inspired oxygen. J Clin Invest 1949;28:1544-1564.Crossref 15. Reinhard EH, Moore CV, Dubach R, et al: Depressant effects of high concentrations of inspired oxygen on erythrocytogenesis: Observations on patients with sickle cell anemia with a description of the observed toxic manifestations of oxygen. J Clin Invest 1944;23:682-698.Crossref 16. Papayannopoulou T, Finch CA: Radioiron measurements of RBC maturation. Blood Cells 1975;1:535-546. 17. Labardini J, Papayannopoulou T, Cook JD, et al: Marrow radioiron kinetics. Haematologia 1973;7:301-312. 18. Wapnick AA, Lunch SR, Krawitz P, et al: Effects of iron overload on ascorbic acid metabolism. Br Med J 1968;3:704-707.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Feb 1, 1982

References