eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Sickle Cell Anemia: Multimedia

Author: Ariel Distenfeld, MD, Clinical Professor, Department of Medicine, New York University School of Medicine
Coauthor(s): Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Contributor Information and Disclosures

Updated: Aug 26, 2009

Multimedia

Molecular and cellular changes of hemoglobin S.Media file 1: Molecular and cellular changes of hemoglobin S.
Molecular and cellular changes of hemoglobin S.

Molecular and cellular changes of hemoglobin S.

Peripheral blood with sickled cells at 400X magni...Media file 2: Peripheral blood with sickled cells at 400X magnification. Image courtesy of Ulrich Woermann, MD.
Peripheral blood with sickled cells at 400X magni...

Peripheral blood with sickled cells at 400X magnification. Image courtesy of Ulrich Woermann, MD.

Peripheral blood smear with sickled cells at 1000...Media file 3: Peripheral blood smear with sickled cells at 1000X magnification. Image courtesy of Ulrich Woermann, MD.
Peripheral blood smear with sickled cells at 1000...

Peripheral blood smear with sickled cells at 1000X magnification. Image courtesy of Ulrich Woermann, MD.

Peripheral blood smear with Howell-Jolly body, in...Media file 4: Peripheral blood smear with Howell-Jolly body, indicating functional asplenism. Image courtesy of Ulrich Woermann, MD.
Peripheral blood smear with Howell-Jolly body, in...

Peripheral blood smear with Howell-Jolly body, indicating functional asplenism. Image courtesy of Ulrich Woermann, MD.

Effects of therapy with hydroxyurea.Media file 5: Effects of therapy with hydroxyurea.
Effects of therapy with hydroxyurea.

Effects of therapy with hydroxyurea.

More on Sickle Cell Anemia

Overview: Sickle Cell Anemia
Differential Diagnoses & Workup: Sickle Cell Anemia
Treatment & Medication: Sickle Cell Anemia
Follow-up: Sickle Cell Anemia
Multimedia: Sickle Cell Anemia
References
Further Reading

References

  1. Adams RJ, McKie VC, Brambilla D, et al. Stroke prevention trial in sickle cell anemia. Control Clin Trials. Feb 1998;19(1):110-29. [Medline].

  2. Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. Jul 2 1998;339(1):5-11. [Medline].

  3. Alikhan M, Carter G, Mehta P. Topical GM-CSF hastens healing of leg ulcers in sickle cell disease. Am J Hematol. Jun 2004;76(2):192. [Medline].

  4. Aslan M, Freeman B. Oxidant-mediated impairment of nitric oxide signaling in sickle cell disease--mechanisms and consequences. Cell Mol Biol (Noisy-le-grand). Feb 2004;50(1):95-105. [Medline].

  5. Ataga K, Sood N, De Gent G, et al. Pulmonary hypertension in sickle cell disease. Am J Med. Nov 1 2004;117(9):665-9. [Medline].

  6. Ballas SK. Complications of sickle cell anemia in adults: guidelines for effective management. Cleve Clin J Med. Jan 1999;66(1):48-58. [Medline].

  7. Ballas SK. Management of sickle pain. Curr Opin Hematol. Mar 1997;4(2):104-11. [Medline].

  8. Beyer JE, Platt AF, Kinney TR, Treadwell M. Practice guidelines for the assessment of children with sickle cell pain. J Soc Pediatr Nurs. Apr-Jun 1999;4(2):61-73. [Medline].

  9. Bookchin RM, Lew VL. Pathophysiology of sickle cell anemia. Hematol Oncol Clin North Am. Dec 1996;10(6):1241-53. [Medline].

  10. Bunn HF. Induction of fetal hemoglobin in sickle cell disease. Blood. Mar 15 1999;93(6):1787-9. [Medline].

  11. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. Sep 11 1997;337(11):762-9. [Medline].

  12. Burnett MW, Bass JW, Cook BA. Etiology of osteomyelitis complicating sickle cell disease. Pediatrics. Feb 1998;101(2):296-7. [Medline].

  13. Castro O. Management of sickle cell disease: recent advances and controversies. Br J Haematol. Oct 1999;107(1):2-11. [Medline].

  14. Charache S. Eye disease in sickling disorders. Hematol Oncol Clin North Am. Dec 1996;10(6):1357-62. [Medline].

  15. Charache S. Mechanism of action of hydroxyurea in the management of sickle cell anemia in adults. Semin Hematol. Jul 1997;34(3 Suppl 3):15-21. [Medline].

  16. Charache S. Treatment of sickling disorders. Curr Opin Hematol. Mar 1996;3(2):139-44. [Medline].

  17. Charache S, Terrin ML, Moore RD, et al. 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. May 18 1995;332(20):1317-22. [Medline].

  18. Chung C, Cackovic M, Kerstein MD. Leg ulcers in patients with sickle cell disease. Adv Wound Care. Sep-Oct 1996;9(5):46-50. [Medline].

  19. Crowley JJ, Sarnaik S. Imaging of sickle cell disease. Pediatr Radiol. Sep 1999;29(9):646-61. [Medline].

  20. Curran EL, Fleming JC, Rice K, Wang WC. Orbital compression syndrome in sickle cell disease. Ophthalmology. Oct 1997;104(10):1610-5. [Medline].

  21. Danzer BI, Birnbach DJ, Thys DM. Anesthesia for the parturient with sickle cell disease. J Clin Anesth. Nov 1996;8(7):598-602. [Medline].

  22. Davies SC. Blood transfusion in sickle cell disease. Curr Opin Hematol. Nov 1996;3(6):485-91. [Medline].

  23. Duncan GW. Ischemic stroke in sickle cell disease: a review. Tenn Med. Dec 1997;90(12):498-9. [Medline].

  24. Eckman JR. Leg ulcers in sickle cell disease. Hematol Oncol Clin North Am. Dec 1996;10(6):1333-44. [Medline].

  25. Elander J, Midence K. A review of evidence about factors affecting quality of pain management in sickle cell disease. Clin J Pain. Sep 1996;12(3):180-93. [Medline].

  26. Ergul S, Brunson CY, Hutchinson J, et al. Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction. Am J Hematol. Jul 2004;76(3):245-51. [Medline].

  27. Faller DV. Endothelial cell responses to hypoxic stress. Clin Exp Pharmacol Physiol. Jan 1999;26(1):74-84. [Medline].

  28. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology. Sep 2004;101(3):766-85. [Medline].

  29. Forbes K, Forbes B, Lee A. Re: Sickle cell-related pain: perceptions of medical practitioners. [letter; comment]. J Pain Symptom Manage. Jun 1998;15(6):333-4. [Medline].

  30. Gebreyohanns M, Adams RJ. Sickle cell disease: primary stroke prevention. CNS Spectr. Jun 2004;9(6):445-9. [Medline].

  31. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. Feb 26 2004;350(9):886-95. [Medline].

  32. Gwilt PR, Tracewell WG. Pharmacokinetics and pharmacodynamics of hydroxyurea. Clin Pharmacokinet. May 1998;34(5):347-58. [Medline].

  33. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood. Mar 1 1997;89(5):1533-42. [Medline].

  34. Hackney AC, Hezier W, Gulledge TP, et al. Effects of hydroxyurea administration on the body weight, body composition and exercise performance of patients with sickle-cell anaemia. Clin Sci (Lond). May 1997;92(5):481-6. [Medline].

  35. Hardwick WE Jr, Givens TG, Monroe KW, et al. Effect of ketorolac in pediatric sickle cell vaso-occlusive pain crisis. Pediatr Emerg Care. Jun 1999;15(3):179-82. [Medline].

  36. Harmatz P, Heyman MB, Cunningham J, et al. Effects of red blood cell transfusion on resting energy expenditure in adolescents with sickle cell anemia. J Pediatr Gastroenterol Nutr. Aug 1999;29(2):127-31. [Medline].

  37. Hillery CA. Potential therapeutic approaches for the treatment of vaso-occlusion in sickle cell disease. Curr Opin Hematol. Mar 1998;5(2):151-5. [Medline].

  38. Hilliard LM, Williams BF, Lounsbury AE, Howard TH. Erythrocytapheresis limits iron accumulation in chronically transfused sickle cell patients. Am J Hematol. Sep 1998;59(1):28-35. [Medline].

  39. Howard LW, Kennedy LD. Hydroxyurea in the treatment of sickle-cell anemia. Ann Pharmacother. Nov 1997;31(11):1393-6. [Medline].

  40. Howlett DC, Hatrick AG, Jarosz JM, et al. The role of CT and MR in imaging the complications of sickle cell disease. Clin Radiol. Nov 1997;52(11):821-9. [Medline].

  41. Jacobson SJ, Kopecky EA, Joshi P, Babul N. Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet. Nov 8 1997;350(9088):1358-61. [Medline].

  42. Kaul DK, Tsai HM, Liu XD, et al. Monoclonal antibodies to alphaVbeta3 (7E3 and LM609) inhibit sickle red blood cell-endothelium interactions induced by platelet-activating factor. Blood. Jan 15 2000;95(2):368-74. [Medline].

  43. Kerle KK, Nishimura KD. Exertional collapse and sudden death associated with sickle cell trait. Mil Med. Dec 1996;161(12):766-7. [Medline].

  44. Kerstein MD. The non-healing leg ulcer: peripheral vascular disease, chronic venous insufficiency, and ischemic vasculitis. Ostomy Wound Manage. Nov-Dec 1996;42(10A Suppl):19S-35S. [Medline].

  45. Kinney TR, Helms RW, O''Branski EE, et al. Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood. Sep 1 1999;94(5):1550-4. [Medline].

  46. Lawrenz DR. Sickle cell disease: a review and update of current therapy. J Oral Maxillofac Surg. Feb 1999;57(2):171-8. [Medline].

  47. Lawton AW. Sickle cell and the eye. ABNF J. May-Jun 1996;7(3):78-80. [Medline].

  48. Lum AF, Wun T, Staunton D, Simon S. Inflammatory potential of neutrophils detected in sickle cell disease. Am J Hematol. Jun 2004;76(2):126-33. [Medline].

  49. Martin JJ, Moore GP. Pearls, pitfalls, and updates for pain management. Emerg Med Clin North Am. May 1997;15(2):399-415. [Medline].

  50. McCarville MB, Li C, Xiong X, Wang W. Comparison of transcranial Doppler sonography with and without imaging in the evaluation of children with sickle cell anemia. AJR Am J Roentgenol. Oct 2004;183(4):1117-22. [Medline].

  51. Meshikhes AW, al-Faraj AA. Sickle cell disease and the general surgeon. J R Coll Surg Edinb. Apr 1998;43(2):73-9. [Medline].

  52. Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. Jan 13 2000;342(2):83-9. [Medline].

  53. Miniero R, Rocha V, Saracco P, et al. Cord blood transplantation (CBT) in hemoglobinopathies. Eurocord. Bone Marrow Transplant. Jul 1998;22 Suppl 1:S78-9. [Medline].

  54. Montgomery KS. Caring for the pregnant woman with sickle cell disease. MCN Am J Matern Child Nurs. Sep-Oct 1996;21(5):224-8. [Medline].

  55. Murray MJ, Evans P. Sudden exertional death in a soldier with sickle cell trait. Mil Med. May 1996;161(5):303-5. [Medline].

  56. Ohene-Frempong K, Smith-Whitley K. Use of hydroxyurea in children with sickle cell disease: what comes next?. Semin Hematol. Jul 1997;34(3 Suppl 3):30-41. [Medline].

  57. Olney RS. Preventing morbidity and mortality from sickle cell disease. A public health perspective. Am J Prev Med. Feb 1999;16(2):116-21. [Medline].

  58. Overturf GD. Infections and immunizations of children with sickle cell disease. Adv Pediatr Infect Dis. 1999;14:191-218. [Medline].

  59. Powars DR, Johnson CS. Priapism. Hematol Oncol Clin North Am. Dec 1996;10(6):1363-72. [Medline].

  60. Quinn CT, Buchanan GR. The acute chest syndrome of sickle cell disease. J Pediatr. Oct 1999;135(4):416-22. [Medline].

  61. Reed W, Vichinsky EP. New considerations in the treatment of sickle cell disease. Annu Rev Med. 1998;49:461-74. [Medline].

  62. Reed WF, Vichinsky EP. Transfusion practice for patients with sickle cell disease. Curr Opin Hematol. Nov 1999;6(6):432-6. [Medline].

  63. Rodgers GP. Overview of pathophysiology and rationale for treatment of sickle cell anemia. Semin Hematol. Jul 1997;34(3 Suppl 3):2-7. [Medline].

  64. Saborio P, Scheinman JI. Sickle cell nephropathy. J Am Soc Nephrol. Jan 1999;10(1):187-92. [Medline].

  65. Schnog JJ, Lard LR, Rojer RA, et al. New concepts in assessing sickle cell disease severity. Am J Hematol. May 1998;58(1):61-6. [Medline].

  66. Serjeant GR. The role of preventive medicine in sickle cell disease. The Watson Smith lecture. J R Coll Physicians Lond. Jan-Feb 1996;30(1):37-41. [Medline].

  67. Shaiova L, Wallenstein D. Outpatient management of sickle cell pain with chronic opioid pharmacotherapy. J Natl Med Assoc. Jul 2004;96(7):984-6. [Medline].

  68. Simon TL, Alverson DC, AuBuchon J, et al. Practice parameter for the use of red blood cell transfusions: developed by the Red Blood Cell Administration Practice Guideline Development Task Force of the College of American Pathologists. Arch Pathol Lab Med. Feb 1998;122(2):130-8. [Medline].

  69. Smith JA. Bone disorders in sickle cell disease. Hematol Oncol Clin North Am. Dec 1996;10(6):1345-56. [Medline].

  70. Steinberg MH. Management of sickle cell disease. N Engl J Med. Apr 1 1999;340(13):1021-30. [Medline].

  71. Steinberg MH. Review: sickle cell disease: present and future treatment. Am J Med Sci. Oct 1996;312(4):166-74. [Medline].

  72. Steinberg MH, Lu ZH, Barton FB, et al. Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea. Blood. Feb 1 1997;89(3):1078-88. [Medline].

  73. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. Oct 9 2004;364(9442):1343-60. [Medline].

  74. Tachakra SS, Davies SC. Management of sickle cell crisis. British Association for Accident and Emergency Medicine guidelines. J Accid Emerg Med. Sep 1998;15(5):356-7. [Medline].

  75. Vermylen C, Cornu G. Bone marrow transplantation for sickle cell anemia. Curr Opin Hematol. Mar 1996;3(2):163-6. [Medline].

  76. Vermylen C, Cornu G, Ferster A, et al. Haematopoietic stem cell transplantation for sickle cell anaemia: the first 50 patients transplanted in Belgium. Bone Marrow Transplant. Jul 1998;22(1):1-6. [Medline].

  77. Vichinsky E, Styles L. Pulmonary complications. Hematol Oncol Clin North Am. Dec 1996;10(6):1275-87. [Medline].

  78. Walters MC, Patience M, Leisenring W, et al. Collaborative multicenter investigation of marrow transplantation for sickle cell disease: current results and future directions. Biol Blood Marrow Transplant. Dec 1997;3(6):310-5. [Medline].

  79. Wick TM, Eckman JR. Molecular basis of sickle cell-endothelial cell interactions. Curr Opin Hematol. Mar 1996;3(2):118-24. [Medline].

  80. Williams R, Olivi S, Li CS, et al. Oral glutamine supplementation decreases resting energy expenditure in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. Oct 2004;26(10):619-25. [Medline].

  81. Wintrobe MM. Lee GL, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Clinical Hematology. Vol 1. 10th ed. Baltimore, Md: Williams & Wilkins;1999: 1329-98.

  82. Zen Q, Batchvarova M, Twyman CA, et al. B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease. Am J Hematol. Feb 2004;75(2):63-72. [Medline].

  83. Roberts L, O'Driscoll S, Dick MC, et al. Stroke prevention in the young child with sickle cell anaemia. Ann Hematol. Oct 2009;88(10):943-6. [Medline].

  84. Rhodes M, Akohoue SA, Shankar SM, et al. Growth patterns in children with sickle cell anemia during puberty. Pediatr Blood Cancer. Oct 2009;53(4):635-41. [Medline].

  85. Al-Subaie AM, Mahdi N, Al-Absi IK, et al. Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis. Eur J Haematol. Aug 21 2009;[Medline].

Further Reading

Related eMedicine Topics

Clinical Trials
National Guideline Clearinghouse

Keywords

sickle cell disease, hemoglobin SS disease, sickle cell trait, homozygous hemoglobin S disease, SCD, mutant hemoglobins, Hb S, Hb SS, Hb A, Hb SA, anemia, red blood cells, RBC, sickle shaped, vasoocclusive crisis, sickle cell crisis, hemoglobin sc disease, hemoglobin c disease, thalassemia, congenital hemolytic anemia, hemoglobinopathies

Contributor Information and Disclosures

Author

Ariel Distenfeld, MD, Clinical Professor, Department of Medicine, New York University School of Medicine
Ariel Distenfeld, MD is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, International Society of Blood Transfusion, International Society of Hematology, Medical Society of the State of New York, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Disclosure: Nothing to disclose.

Medical Editor

Wadie F Bahou, MD, Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook
Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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