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Sickle cell diseases: current therapeutic options and potential pitfalls in preventive therapy for transcranial Doppler abnormalities

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Abstract

Sickle cell disease is an important and common hemoglobinopathy that is highly prevalent worldwide. Recent clinical research has clarified the natural history, and newer, exciting therapeutic maneuvers have been developed, including stem cell transplantation, a curative, albeit toxic strategy. There is a need for early identification of a severe disease profile so that these newer therapeutic interventions can be offered before severe organ damage occurs. Investigative methodologic research by radiologists to discover early organ damage can be important to successful planning of treatment protocols. Stroke is one complication, which, if diagnosed early, can be satisfactorily managed with more aggressive therapy. The advent of transcranial Doppler and MRI have greatly increased the ability to detect early CNS disease.

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References

  1. Herrick J (1910) Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Int Med 6:517–521

    Google Scholar 

  2. Lee A, Thomas P, Cupidore L, et al (1995) Improved survival in homozygous sickle cell disease: lessons from a cohort study. BMJ 311:1600–1602

    Google Scholar 

  3. Quinn CT, Rogers ZR, Buchanan GR (2004) Survival of children with sickle cell disease. Blood 103:4023–4027

    Article  Google Scholar 

  4. Leikin SL, Gallagher D, Kinney TR, et al (1989) Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatrics 84:500–508

    Google Scholar 

  5. Brown AK, Sleeper LA, Miller ST, et al (1994) Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med 148:796–804

    Google Scholar 

  6. Platt OS, Thorington BD, Brambilla DJ, et al (1991) Pain in sickle cell disease. Rates and risk factors. N Engl J Med 325:11–16

    CAS  PubMed  Google Scholar 

  7. Miller ST, Sleeper LA, Pegelow CH, et al (2000) Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 342:83–89

    Article  CAS  PubMed  Google Scholar 

  8. Rucknagel DL (2001) The role of rib infarcts in the acute chest syndrome of sickle cell diseases. Pediatr Pathol Mol Med 20:137–154

    Article  Google Scholar 

  9. Charache S, Terrin ML, Moore RD, et al (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med 332:1317–1322

    Article  Google Scholar 

  10. Charache S, Barton FB, Moore RD, et al. (1996) Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive “switching” agent. The multicenter study of hydroxyurea in sickle cell anemia. Medicine (Baltimore) 75:300–326

    Article  Google Scholar 

  11. Mentzer WC, Heller S, Pearle PR, et al. (1994) Availability of related donors for bone marrow transplantation in sickle cell anemia. Am J Pediatr Hematol Oncol 16:27–29

    Google Scholar 

  12. Walters MC, Storb R, Patience M, et al (2000) Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease. Blood 95:1918–1924

    Google Scholar 

  13. Walters MC, Patience M, Leisenring W, et al (2001) Stable mixed hematopoietic chimerism after bone marrow transplantation for sickle cell anemia. Biol Blood Marrow Transplant 7:665–673

    Article  Google Scholar 

  14. Krishnamurti L, Blazar BR, Wagner JE (2001) Bone marrow transplantation without myeloablation for sickle cell disease. N Engl J Med 344:68

    Article  CAS  Google Scholar 

  15. Adams GT, Snieder H, McKie VC, et al. (2003) Genetic risk factors for cerebrovascular disease in children with sickle cell disease: design of a case-control association study and genomewide screen. BMC Med Genet 4:6

    Article  Google Scholar 

  16. Vichinsky EP, Johnson R, Lubin BH (1982) Multidisciplinary approach to pain management in sickle cell disease. Am J Pediatr Hematol Oncol 4:328–333

    Google Scholar 

  17. Sarnaik SA, Ballas SK (2001) Molecular characteristics of pediatric patients with sickle cell anemia and stroke. Am J Hematol 67:179–182

    Article  Google Scholar 

  18. Nagel RL, Steinberg MH, el-Hazmi MA, et al. (2001) Role of epistatic (modifier) genes in the modulation of the phenotypic diversity of sickle cell anemia on the molecular interactions between alpha-thalassemia and sickle cell gene. The interaction of alpha-thalassemia and homozygous sickle-cell disease. Concurrent sickle-cell anemia and alpha-thalassemia: effect on severity of anemia. Pediatr Pathol Mol Med 20:123–136

    Article  Google Scholar 

  19. Kasschau MR, Barabino GA, Bridges KR, et al (1996) Adhesion of sickle neutrophils and erythrocytes to fibronectin. Blood 87:771–780

    Google Scholar 

  20. Sugihara K, Sugihara T, Mohandas N, et al (1992) Thrombospondin mediates adherence of CD36+ sickle reticulocytes to endothelial cells. Blood 80:2634–2642

    Google Scholar 

  21. Francis RB Jr (1991) Platelets, coagulation, and fibrinolysis in sickle cell disease: their possible role in vascular occlusion. Blood Coagul Fibrinolysis 2:341–353

    Google Scholar 

  22. Powars D, Wilson B, Imbus C, et al (1978) The natural history of stroke in sickle cell disease. Am J Med 65:461–471

    Article  Google Scholar 

  23. Russell MO, Goldberg HI, Reis L, et al (1976) Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. J Pediatr 88:382–387

    Google Scholar 

  24. Sarnaik S, Soorya D, Kim J, et al (1979) Periodic transfusions for sickle cell anemia and CNS infarction. Am J Dis Child 133:1254–1257

    Google Scholar 

  25. Pegelow CH, Adams RJ, McKie V, et al (1995) Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 126:896–899

    CAS  PubMed  Google Scholar 

  26. Wang WC, Kovnar EH, Tonkin IL, et al (1991) High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. J Pediatr 118:377–382

    Google Scholar 

  27. Sarnaik S, Schornack J, Lusher JM (1986) The incidence of development of irregular red cell antibodies in patients with sickle cell anemia. Transfusion 26:249–252

    Article  Google Scholar 

  28. Rosse WF, Gallagher D, Kinney TR, et al (1990) Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease. Blood 76:1431–1437

    Google Scholar 

  29. Sarnaik S CC-H, Lusher J (1989) Severe liver disease with chronic transfusion in sickle cell disease: mortality and morbidity. Pediatr Res 25:156a

    Google Scholar 

  30. Adams R, McKie V, Nichols F, et al (1992) The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 326:605–610

    Google Scholar 

  31. Adams RJ, McKie VC, Carl EM, et al (1997) Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Ann Neurol 42:699–704

    CAS  PubMed  Google Scholar 

  32. Adams RJ, McKie VC, Hsu L, et al (1998) Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 339:5–11

    Article  CAS  PubMed  Google Scholar 

  33. Adams RJ, Brambilla DJ, Granger S, et al (2004) Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study. Blood 103:3689–3694

    Article  CAS  PubMed  Google Scholar 

  34. (2002) Health supervision for children with sickle cell disease. Pediatrics 109:526–535

  35. Lane P, Buchanan G, Ware R (2001) Variable approaches to therapeutic options for children with sickle cell disease: a practice survey of the American Society of Pediatric Hematology/Oncology (ASPH/O). Blood 98:784a

    Google Scholar 

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Authors and Affiliations

  1. The Carman and Ann Adams Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA

    Sharada A. Sarnaik

  2. Director, Sickle Cell Center, Children’s Hospital of Michigan, 3901 Beaubien Blvd., Detroit, 48201, MI, USA

    Sharada A. Sarnaik

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  1. Sharada A. Sarnaik
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Correspondence to Sharada A. Sarnaik.

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Sarnaik, S.A. Sickle cell diseases: current therapeutic options and potential pitfalls in preventive therapy for transcranial Doppler abnormalities. Pediatr Radiol 35, 223–228 (2005). https://doi.org/10.1007/s00247-005-1408-8

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  • DOI: https://doi.org/10.1007/s00247-005-1408-8

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