Seminars in hematology
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The expanding indications for transfusions in patients with sickle cell disease raise the issues of appropriate measurement of body iron burden and optimal timing of iron chelation therapy. In this study, we obtained 42 biopsy specimens from 20 patients with sickle cell disease (mean age, 15.7 years) who received transfusions. In 12 patients whose mean age was 11.3 years at the time of liver biopsy, hepatic iron concentration was measured to provide information about the rate of iron accumulation in sickle cell disease, as well as to guide the initiation of chelating therapy. ⋯ These data show that after 1 to 2 years of conventional transfusions, variable tissue iron concentrations and tissue damage are observed in patients with sickle cell disease. In some patients, iron chelation therapy may not be appropriate after 1 year of transfusions; in others, therapy is clearly indicated by this time to prevent tissue injury. The data also suggest that patients with sickle cell disease develop increased portal fibrosis at the thresholds previously described in young patients with thalassemia (approximately 7 mg/g of liver, dry weight).
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Myeloproliferative diseases (MPD) include polycythemia vera (PV), essential thrombocythemia, agnogenic myeloid metaplasia, and chronic myelogenous leukemia. The focus of this report is on PV, which is characterized by an increase in red blood cells, granulocytes, and platelets. Complications associated with PV are an increased risk of thrombosis and abnormal bleeding. ⋯ It is clear that no single agent satisfies all the needs for cytoreduction that arise during the course of PV. Future protocols should be designed that draw on the large body of experience already gained with these drugs to transcend the limitations of single-agent therapy and to improve quality of life as well as survival. Semin Hematol 38(suppl 2):25-28.
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Seminars in hematology · Jan 2001
Multicenter StudyIron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease.
Patients with sickle cell disease (SCD) often require blood transfusion starting in early childhood. Multiple blood transfusions on a chronic basis lead to excessive accumulation of iron, especially in adults with sickle cell anemia (SS) that is progressively increasing in size. Blood exchange transfusion and the use of iron chelation therapy may prevent or delay the onset of iron overload. ⋯ Patients with low values of serum ferritin and % Sat had lower incidence of acute painful episodes (38% v 64%) and organ failure (19% v 71%) than those who had iron overload, respectively. Mortality was significantly higher in the iron overload group: 64% versus 5%, respectively. Taken together, the data indicate that (1) the status of iron stores in adults with SS is best determined by keeping accurate records of the amount of blood transfused and serial determinations of ferritin levels in the steady state; (2) a significant number of adults with SS have iron overload; and (3) iron overload seems to be a predisposing factor of disease severity.
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Seminars in hematology · Jan 2001
ReviewCurrent issues with blood transfusions in sickle cell disease.
With increased recognition of the profound morbidity of sickle cell disease and with growing evidence of the efficacy of transfusion therapy in prevention and treatment of sickle cell complications, most patients now receive intermittent transfusion therapy. The purpose of this report is to review blood component therapy and Its risks for sickle cell patients. Packed red cells are the preferred blood component. ⋯ In part, these are induced by blood viscosity and increased blood pressure. Diuretic therapy and close monitoring of transfusion volume and vital signs can minimize these events. In summary, transfusion therapy carries risks, but the routine use of leukocyte-reduced, phenotypically matched units in conjunction with close monitoring of patients can make transfusion therapy safer.