Anemia de células falciformes
Sickle cell anemia
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Chaves, W., Amador, D., & Sánchez, J. (2014). Anemia de células falciformes. Revista Repertorio De Medicina Y Cirugía, 23(3), 221–225. https://doi.org/10.31260/RepertMedCir.v23.n3.2014.715
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Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-CompartirIgual 4.0.
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Presentamos dos mujeres que consultaron a urgencias por dolor abdominal agudo, iniciado pocas horas después de su llegada a Bogotá DC, asociado con síndrome anémico y hallazgos paraclínicos sugestivos de anemia de células falciformes, que se confirmó con electroforesis de proteínas en la consulta externa. La drepanocitosis, conocida como anemia de células falciformes, es una hemoglobinopatía estructural de causa genética que ocasiona clínica vasoclusiva con isquemia tisular secundaria generadora de crisis de dolor, que se exacerba entre otras razones por cambios de temperatura y de la presión del oxígeno atmosférico. Es importante causa de dolor agudo en pacientes de raza negra que asisten a urgencias al provenir con frecuencia de lugares cálidos.
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2. Bender MA, Hobbs W. Sickle cell disease. In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, Fong CT, et al. GeneReviewsTM[monografía en Internet]. Washington, Seattle; 1993-2014 [citado 9 Jul 2014]. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK1377/
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5. Lane PA, O’Connell JL, Lear JL, Rogers ZR, Woods GM, Hassell KL, et al. Functional asplenia in hemoglobin SC disease. Blood. 1995;85(8):2238-44.
6. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009;122(6):507-12.
7. Gong L, Parikh S, Rosenthal PJ, Greenhouse B. Biochemical and immunological mechanisms by which sickle cell trait protects against malaria. Malar J. 2013;12(1):317.
8. Durán CL, Morales OL, Echeverri SJ, Isaza M. Haplotipos del gen de la globina beta en portadores de hemoglobina S en Colombia. Biomedica. 2012;32(1):103-11.
9. Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med. 1986;80(5):985-90.
10. Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med. 1978;65(3):461-71.
11. Minter KR, Gladwin MT. Pulmonary complications of sickle cell anemia. A need for increased recognition, treatment, and research. Am J Respir Crit Care Med. 2001;164(11):2016-9.
12. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010;85(1):6-13.
13. Nagalla S, Ballas SK. Drugs for preventing red blood cell dehydration in people with sickle cell disease. Cochrane Database Syst Rev. 2012;7:CD003426.
14. Vichinsky E, Williams R, Das M, Earles AN, Lewis N, Adler A, et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood. 1994;83(11):3107-12.
15. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855-65.
16. National Institute of Health, National Heart Lung and Blood Institute. The management of sickle cell disease [monografía en Internet]. Washington: The Institute; 2004 [citado 9 Jul 2014]. Disponible en: http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf
17. Davies S, Olujohungbe A. Hydroxyurea for sickle cell disease. Cochrane Database Syst Rev. 2001(2):CD002202.
18. Segal JB, Strouse JJ, Beach MC, et al. Hydroxyurea for the treatment of sickle cell disease [monografía en Internet]. Rockville (MD): Agency for Healthcare Research and Quality; 2008 [citado 9 Jul 2014]. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK38503/
19. Halsey C, Roberts IA. The role of hydroxyurea in sickle cell disease. Br J Haematol. 2003;120(2):177-86.
20. Platt OS. Hydroxyurea for the treatment of sickle cell anemia. N Engl J Med. 2008;358(13):1362-9.
21. Ortega Aramburu JJ. [Sickle-cell anemia: an emerging disease in Spain]. An Pediatr (Barc). 2003;58(2):93-4.
22. Wilson RE, Krishnamurti L, Kamat D. Management of sickle cell disease in primary care. Clin Pediatr (Phila). 2003;42(9):753-61.
23. Ruggeri A, Eapen M, Scaravadou A, Cairo MS, Bhatia M, Kurtzberg J, et al. Umbilical cord blood transplantation for children with thalassemia and sickle cell disease. Biol Blood Marrow Transplant. 2011;17(9):1375-82.
2. Bender MA, Hobbs W. Sickle cell disease. In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, Fong CT, et al. GeneReviewsTM[monografía en Internet]. Washington, Seattle; 1993-2014 [citado 9 Jul 2014]. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK1377/
3. Denis M. Sickle cell anemia. In: Miller LP, editor. Blood diseases of infancy and childhood. 7th ed. St. Louis, Toronto: Mosby; 1995. p. 415.
4. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-44.
5. Lane PA, O’Connell JL, Lear JL, Rogers ZR, Woods GM, Hassell KL, et al. Functional asplenia in hemoglobin SC disease. Blood. 1995;85(8):2238-44.
6. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009;122(6):507-12.
7. Gong L, Parikh S, Rosenthal PJ, Greenhouse B. Biochemical and immunological mechanisms by which sickle cell trait protects against malaria. Malar J. 2013;12(1):317.
8. Durán CL, Morales OL, Echeverri SJ, Isaza M. Haplotipos del gen de la globina beta en portadores de hemoglobina S en Colombia. Biomedica. 2012;32(1):103-11.
9. Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med. 1986;80(5):985-90.
10. Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med. 1978;65(3):461-71.
11. Minter KR, Gladwin MT. Pulmonary complications of sickle cell anemia. A need for increased recognition, treatment, and research. Am J Respir Crit Care Med. 2001;164(11):2016-9.
12. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010;85(1):6-13.
13. Nagalla S, Ballas SK. Drugs for preventing red blood cell dehydration in people with sickle cell disease. Cochrane Database Syst Rev. 2012;7:CD003426.
14. Vichinsky E, Williams R, Das M, Earles AN, Lewis N, Adler A, et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood. 1994;83(11):3107-12.
15. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855-65.
16. National Institute of Health, National Heart Lung and Blood Institute. The management of sickle cell disease [monografía en Internet]. Washington: The Institute; 2004 [citado 9 Jul 2014]. Disponible en: http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf
17. Davies S, Olujohungbe A. Hydroxyurea for sickle cell disease. Cochrane Database Syst Rev. 2001(2):CD002202.
18. Segal JB, Strouse JJ, Beach MC, et al. Hydroxyurea for the treatment of sickle cell disease [monografía en Internet]. Rockville (MD): Agency for Healthcare Research and Quality; 2008 [citado 9 Jul 2014]. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK38503/
19. Halsey C, Roberts IA. The role of hydroxyurea in sickle cell disease. Br J Haematol. 2003;120(2):177-86.
20. Platt OS. Hydroxyurea for the treatment of sickle cell anemia. N Engl J Med. 2008;358(13):1362-9.
21. Ortega Aramburu JJ. [Sickle-cell anemia: an emerging disease in Spain]. An Pediatr (Barc). 2003;58(2):93-4.
22. Wilson RE, Krishnamurti L, Kamat D. Management of sickle cell disease in primary care. Clin Pediatr (Phila). 2003;42(9):753-61.
23. Ruggeri A, Eapen M, Scaravadou A, Cairo MS, Bhatia M, Kurtzberg J, et al. Umbilical cord blood transplantation for children with thalassemia and sickle cell disease. Biol Blood Marrow Transplant. 2011;17(9):1375-82.
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