medigraphic.com
Colegio de Medicina Interna de México.

2024, Number 9

<< Back Next >>

Med Int Mex 2024; 40 (9)

Sickle cell disease in emergency department: An evidence-based review

Duque EL, Saavedra VME, Vergara YD, Martínez-Sánchez LM

Language: Spanish
References: 58
Page: 581-590
PDF size: 358.00 Kb.


ABSTRACT

Sickle cell disease is an autosomal recessive hematological disorder caused by an amino acid substitution in the beta-globin chain of adult hemoglobin. This substitution produces polymerization of hemoglobin resulting in sickle-shaped red blood cells, this process in addition to generating rigid erythrocytes initiates a series of events that include changes in the function of the erythrocyte membrane, uniform distribution of erythrocyte volume, alterations in endothelial activity and increased adherence to the vascular endothelium, uniform distribution of erythrocyte volume, alterations in endothelial activity and increased adherence to the vascular endothelium, causing complications such as vaso-occlusive crises, pulmonary hypertension, chronic pain, ischemic complications, anemia, pneumococcal infections and acute chest syndrome. Clinically the disease is characterized by hemolytic anemia, which progressively occludes different organs, with vaso-occlusive crises and pain. Pain is the most frequent symptom and the one that generates most costs to the health system, due to emergency department visits and number of hospitalizations. These pain crises are of variable duration and can occur at different sites of the body, but most often in bones like the spine, lower extremities in adults and dactylitis in infants or newborns. The global treatment for sickle cell disease is based on the use of hydroxyurea, red blood cell transfusion, erythroapheresis, stem cell transplantation in selected patients and recently glutamine has appeared as a new choice.


REFERENCES

  1. Weaver SB, Rungkitwattanakul D, Singh D. Contemporarymanagement and prevention of vaso-occlusivecrises (CVOs) in adults with sickle cell disease. JPharm Pract 2021: 8971900211026644. https://doi.or/10.1177/08971900211026644

  2. Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, etal. Management of sickle cell disease: summary of the2014 evidence-based report by expert panel members.JAMA 2014; 312 (10): 1033-48. https://doi.or/10.1001/jama.2014.10517.

  3. Piel FB, Steinberg MH, Rees DC. Sickle Cell Disease. N EnglJ Med 2017; 376 (16): 1561-73. https://doi.or/10.1056/NEJMra151086

  4. Tran H, Gupta M, Gupta K. Targeting novel mechanisms ofpain in sickle cell disease. Blood 2017; 130 (22): 2377-85.https://doi.or/10.1182/blood-2017-05-78200

  5. Ingram VM. A specific chemical difference between theglobins of normal human and sickle-cell anaemia haemoglobin.Nature 1956; 178 (4537): 792-94. https://doi.or/10.1038/178792a0

  6. Telen MJ, Malik P, Vercellotti GM. Therapeutic strategies forsickle cell disease: towards a multi-agent approach. Nat RevDrug Discov 2019; 18 (2): 139-58. https://doi.or/10.1038/s41573-018-0003-2

  7. Sundd P, Gladwin MT, Novelli EM. Pathophysiology of SickleCell Disease. Annu Rev Pathol 2019; 14: 263-92. https://doi.or/10.1146/annurev-pathmechdis-012418-012838

  8. Sickle Cell Disease (ACF). Centers for disease control andprevention. Publicado en diciembre 16, 2020. www.cdc.gov/ncbddd/sicklecell/data.htm

  9. GBD 2013 Mortality and Causes of Death Collaborators.Global, regional, and national age-sex specific all-causeand cause-specific mortality for 240 causes of death, 1990-2013: a systematic analysis for the Global Burden of DiseaseStudy 2013. Lancet 2015; 385 (9963): 117-71. https://doi.or/10.1016/S0140-6736(14)61682-2

  10. Kauf TL, Coates TD, Huazhi L, Mody-Patel N, et al. Thecost of health care for children and adults with sickle celldisease. Am J Hematol 2009; 84 (6): 323-27. https://doi.or/10.1002/ajh.21408

  11. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease.Lancet 2010; 376 (9757): 2018-31. https://doi.or/10.1016/S0140-6736(10)61029-X

  12. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, et al; Investigators,Comprehensive Sickle Cell Centers. Definitions of thephenotypic manifestations of sickle cell disease. Am J Hematol2010; 85 (1): 6-13. https://doi.or/10.1002/ajh.21550

  13. McCavit TL. Sickle cell disease. Pediatr Rev 2012; 33 (5):195-204. https://doi.or/10.1542/pir.33-5-195

  14. Quinn CT. Sickle cell disease in childhood: from newbornscreening through transition to adult medical care. PediatrClin North Am 2013; 60 (6): 1363-81. https://doi.or/10.1016/j.pcl.2013.09.006

  15. Pace BS, Starlard-Davenport A, Kutlar A. Sickle cell disease:progress towards combination drug therapy. Br J Haematol2021; 194 (2): 240-51. https://doi.or/10.1111/bjh.17312

  16. Weatherall DJ. The role of the inherited disorders of hemoglobin,the first “molecular diseases” in the future of humangenetics. Annu Rev Genomics Hum Genet 2013; 14: 1-24.https://doi.or/10.1146/annurev-genom-091212-153500

  17. Serjeant GR. The natural history of sickle cell disease. ColdSpring Harb Perspect Med 2013; 3 (10): a011783. https://doi.or/10.1101/cshperspect.a011783

  18. Bouchán-Valencia P, Coeto-Barona G, Rosenfeld-MannF, Trueba-Gómez R, et al. Identificación molecular de lahemoglobina D Punjab en dos familias. Revista Médica delInstituto Mexicano del Seguro Social 2016; 54 (6): 793-800.https://www.redalyc.org/articulo.oa?id=45774791801

  19. Ruiz-Argüelles GJ, López-Martı́nez B, Ruiz-Reyes G. Heterozygousβ-Thalassemia: Not Infrequent in Mexico. ArchMed Res 2001; 34 (4): 293-95. https://doi.org/10.1016/S0188-4409(01)00284

  20. Perea FJ, Casas-Castañeda M, Villalobos-ArámbulaAR, Barajas H, et al. Hb D-Los Angeles associated withHb S or beta-thalassemia in four Mexican Mestizofamilies. Hemoglobin 1999; 23 (3): 231-37. https://doi.or/10.3109/03630269909005703

  21. Pinto VM, Balocco M, Quintino S, Forni GL. Sickle cell disease:a review for the internist. Intern Emerg Med 2019; 14(7): 1051-64. https://doi.or/10.1007/s11739-019-02160-x

  22. Hequet O, Fort R, Driss F. Red blood cell exchange in anemergency in sickle cell disease. Transfus Apher Sci 2020; 59(6): 102996. https://doi.or/10.1016/j.transci.2020.102996

  23. Lovett PB, Sule HP, Lopez BL. Sickle cell disease in the EmergencyDepartment. Hematol Oncol Clin North Am 2017; 31(6): 1061-79. https://doi.or/10.1016/j.hoc.2017.08.009

  24. Alayash AI. Oxidative pathways in the sickle cell andbeyond. Blood Cells Mol Dis 2018; 70: 78-86. https://doi.or/10.1016/j.bcmd.2017.05.009

  25. Shah F, Dwivedi M. Pathophysiology and recent therapeuticinsights of sickle cell disease. Ann Hematol 2020; 99 (5):925-35. https://doi.or/10.1007/s00277-020-03977-9

  26. Correa Saavedra MA. Anemia de células falciformes: correlaciónclínico-patológica. Archivos de Medicina (Col)2019; 19 (1): 160-67.

  27. Shah N, Bhor M, Xie L, Paulose J, et al. Sickle cell diseasecomplications: Prevalence and resource utilization. PLoSOne 2019; 14 (7): e0214355. https://doi.or/10.1371/journal.pone.0214355

  28. Glassberg JA. Improving Emergency Department-BasedCare of Sickle Cell Pain. Hematology Am Soc Hematol EducProgram 2017; 2017 (1): 412-17. https://doi.or/10.1182/asheducation-2017.1.412

  29. Zúñiga P, Martínez C, Gónzalez LM, Rendón DS, et al. Enfermedadde células falciformes: un diagnóstico para tenerpresente. Rev Chil Pediatr 2018; 89 (4): 525-29.

  30. Tiam L, Dramé A, Coly IZ, Diouf FN, et al. Epidemiological,clinical and hematological profiles of homozygous sickle celldisease during the intercritical period among children inZiguinchor, Senegal. Pan Afr Med J 2017; 28: 208. https://doi.or/10.11604/ pamj.2017.28.208.14006

  31. Farooq S, Testai FD. Neurologic Complications of Sickle CellDisease. Curr Neurol Neurosci Rep 2019; 19 (4): 17. https://doi.or/10.1007/s11910-019-0932-0

  32. Maioli MC, Soares AR, Bedirian R, Alves UD, et al. Relationshipbetween pulmonary and cardiac abnormalitiesin sickle cell disease: implications for the management ofpatients. Rev Bras Hematol Hemoter 2016; 38 (1): 21-7.https://doi.or/10.1016/j.bjhh.2015.11.001

  33. Ballas SK. Sickle cell disease: Classification of clinical complicationsand approaches to preventive and therapeuticmanagement. Clin Hemorheol Microcirc 2018; 68 (2-3):105-28. https://doi.or/10.3233/CH-189002

  34. Onimoe G, Rotz S. Sickle cell disease: A primary careupdate. Cleve Clin J Med 2020; 87 (1): 19-27. https://doi.or/10.3949/ccjm.87a.18051

  35. Wautier JL, Wautier MP. Cellular and molecular aspects ofblood cell-endothelium interactions in vascular disorders.Int J Mol Sci 2020; 21 (15): 5315. https://doi.or/10.3390/ijms21155315

  36. Lee S, Lucas S, Proudman D, Nellesen D, et al. Burden ofcentral nervous system complications in sickle cell disease:A systematic review and meta-analysis. Pediatr BloodCancer 2022; 69 (4): e29493. https://doi.or/10.1002/pbc.29493

  37. Ballas SK, Lusardi M. Hospital readmission for adult acutesickle cell painful episodes: frequency, etiology, and prognosticsignificance. Am J Hematol 2005; 79 (1): 17-25.https://doi.or/10.1002/ajh.20336

  38. Ramsay Z, Bartlett R, Ali A, Grant J, et al. Sickle CellDisease and Pain: Is it all Vaso-occlusive Crises? ClinJ Pain 2021; 37 (8): 583-90. https://doi.or/10.1097/AJP.0000000000000949

  39. Field JJ, Ballas SK, Campbell CM, Crosby LE, et al. AAAPTDiagnostic Criteria for Acute Sickle Cell Disease Pain.J Pain 2019; 20 (7): 746-59. https://doi.or/10.1016/j.jpain.2018.12.003

  40. Ballas SK, Smith ED. Red blood cell changes during theevolution of the sickle cell painful crisis. Blood 1992; 79(8): 2154-63.

  41. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: acritical reappraisal. Blood. 2012 Nov 1;120(18):3647-56.https://doi.org/10.1182/blood-2012-04-383430

  42. Arnáez Solís J, Ortega Molina M, Cervera Bravo A, RoaFrancia MA, et al. Evaluación de veintitrés episodios desíndrome torácico agudo en pacientes con drepanocitosis.An Pediatr (Barc) 2005; 62 (3): 221-8. https://doi.org/10.1157/13071836

  43. Naik RP, Smith-Whitley K, Hassell KL, Umeh NI, de MontalembertM, Sahota P, et.al. Clinical outcomes associatedwith sickle cell trait: a systematic review. Ann InternMed 2018; 169 (9): 619-627. https://doi.org/10.7326/M18-1161

  44. Whipple NS, Joshi VM, Naik RJ, Mentnech T, et al. Sicklecell disease and ventricular myocardial strain: A systematicreview. Pediatr Blood Cancer 2021; 68 (6): e28973. https://doi.org/1002/pbc.28973

  45. Wagdy R, Suliman H, Bamashmose B, Aidaroos A, et al.Subclinical myocardial injury during vaso-occlusive crisisin pediatric sickle cell disease. Eur J Pediatr 2018; 177(12): 1745-52. https://doi.org/10.1007/s00431-018-3231-x

  46. Lawrence C, Webb J. Sickle cell disease and stroke: diagnosisand management. Curr Neurol Neurosci Rep 2016;16 (3): 27. https://doi.org/10.1007/s11910-016-0622-0

  47. Noubiap JJ, Mengnjo MK, Nicastro N, Kamtchum-TatueneJ. Neurologic complications of sickle cell disease inAfrica: A systematic review and meta-analysis. Neurology2017; 89 (14): 1516-24. https://doi.org/10.1212/WNL.0000000000004537

  48. Almeida A, Roberts I. Bone involvement in sickle celldisease. Br J Haematol 2005; 129 (4): 482-90. https://doi.org/10.1111/j.1365-2141.2005.05476.x

  49. Ballas SK, Darbari DS. Review/overview of pain in sickle celldisease. Complement Ther Med 2020; 49: 102327. https://doi.org/1016/j.ctim.2020.102327

  50. Chinegwundoh FI, Smith S, Anie KA. Treatments for priapismin boys and men with sickle cell disease. CochraneDatabase Syst Rev 2020; 4 (4): CD004198. https://doi.org/10.1002/14651858

  51. Ahuja G, Ibecheozor C, Okorie NC, Jain AJ, et al. Priapismand sickle cell disease: special considerations in etiology,management, and prevention. Urology 2021; 156: e40-e47.https://doi.org/10.1016/j.urology.2021.06.010

  52. Arduini GAO, Trovó de Marqui AB. Prevalence and characteristicsof priapism in sickle cell disease. Hemoglobin2018; 42 (2): 73-77. https://doi.org/10.1080/03630269.2018.1452760

  53. Brandow AM, Carroll CP, Creary S, Edwards-Elliott R, et al.American Society of Hematology 2020 guidelines for sicklecell disease: management of acute and chronic pain. BloodAdv 2020; 4 (12): 2656-2701. https://doi.org/10.1182/bloodadvances.2020001851

  54. Hoppe C, Neumayr L. Sickle cell disease: monitoring, currenttreatment, and therapeutics under development. HematolOncol Clin North Am 2019; 33 (3): 355-371. https://doi.org/10.1016/j.hoc.2019.01.014

  55. Meier ER. Treatment options for sickle cell disease.Pediatr Clin North Am 2018; 65 (3): 427-43. https://doi.org/10.1016/j.pcl.2018.01.005

  56. Tisdale JF, Thein SL, Eaton WA. Treating sickle cell anemia.Science 2020; 367 (6483): 1198-99. https://doi.org/10.1126/science. aba3827

  57. Williams TN, Thein SL. Sickle cell anemia and its phenotypes.Annu Rev Genomics Hum Genet 2018; 19: 113-147.https://doi.org/10.1146/annurev-genom-083117-021320

  58. Darbari DS, Sheehan VA, Ballas SK. The vaso-occlusive paincrisis in sickle cell disease: Definition, pathophysiology,and management. Eur J Haematol 2020; 105 (3): 237-46.https://doi.org/10.1111/ejh.13430




2020     |     www.medigraphic.com