Sickle Cell Diseases

Abstract

Sickle cell diseases (SCDs) are chronic destructive process on endothelium particularly at the capillary level. The chronic catastrophic process initiates at birth, and terminates with accelerated endotelial damage induced multiorgan failures in early years of life. Chronic obstructive pulmonary disease, leg ulcers, digital clubbing, priapism, coronary heart disease, chronic renal disease, peripheric artery disease, cirrhosis, and stroke are just some of the terminal end points of the diseases. Red blood cell transfusions should be the treatment of choice nearly in all acute events and emergencies of the diseases to prevent disseminated tissue hypoxia all over the body. In long term treatment, an oral hydroxyurea is cheap, safe, and effective drug, not to prevent but to delay the end points of the diseases.

Key Words: Sickle cell diseases, chronic endothelial inflammation

Keywords

• Sickle cell diseases, chronic endothelial inflammation

Orak Hücre Hastalıkları

Abstract

Orak hücre hastalıkları (OHH), damar endoteli üzerine uzun süreli, yıkıcı bir süreçtir ve özellikle kılcal damar ağı düzeyinde hasara neden olur. Bu uzun süreli ve yıkıcı süreç doğumda başlar ve yaşamın erken dönemlerinde hızlanmış endotel hasarına bağlı çoklu organ yetmezlikleriyle sonlanır. Kronik tıkayıcı akciğer hastalığı, bacak yarası, çomaklaşma, priapizm, koroner kalp hastalığı, kronik böbrek hastalığı, çevresel damar hastalığı, siroz ve inme muhtemelen OHH’de sonlanma noktalarından sadece birkaçıdır. OHH’de gelişebilecek neredeyse tüm akut olaylar ve acillerde vücutta gelişen yaygın doku hipoksisinin önlenmesinde etkili tedavi yöntemi kırmızı küre desteği olmalıdır. Uzun dönem tedavide, OHH’de gelişebilecek sonlanma noktalarının tümüyle önlenmesinde olmasa da geciktirilmesinde ağızdan alınan hidroksiüre ucuz, güvenli ve etkili bir ilaçtır.

Anahtar Kelimeler: Orak hücre hastalıkları, kronik endotel iltihabı

Keywords

• Orak hücre hastalıkları, kronik endotel iltihabı

References

1. Helvaci MR, Aydogan A, Akkucuk S, Oruc C, Ugur M. Sickle cell diseases and ileus. Int J Clin Exp Med 2014;7:2871-2876.
2. Helvaci MR, Acipayam C, Aydogan A, Akkucuk S, Oruc C, Gokce C. Acute chest syndrome in severity of sickle cell diseases. Int J Clin Exp Med 2014;7:5790-5795.
3. Helvaci MR, Aydin Y, Ayyildiz O. Hydroxyurea may prolong survival of sickle cell patients by decreasing frequency of painful crises. HealthMED 2013;7:2327-2332.
4. Helvaci MR, Gokce C, Davran R, Acipayam C, Akkucuk S, Ugur M. Tonsilectomy in sickle cell diseases. Int J Clin Exp Med 2015;8:4586-4590.
5. Helvaci MR, Erden ES, Aydin LY. Atherosclerotic background of chronic obstructive pulmonary disease in sickle cell patients. HealthMed 2013;7:484-488.
6. 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;330:1639-1644.
7. Helvaci MR, Ayyildiz O, Gundogdu M. Gender differences in severity of sickle cell diseases in non-smokers. Pak J Med Sci 2013;29:1050-1054.
8. Parfrey NA, Moore W, Hutchins GM. Is pain crisis a cause of death in sickle cell disease? Am J Clin Pathol 1985;84:209-212.

9. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 2000;342:83-89.
10. Balkaran B, Char G, Morris JS, Thomas PW, Serjeant BE, Serjeant GR. Stroke in a cohort of patients with homozygous sickle cell disease. J Pediatr 1992;120:360-366.
11. Cole TB, Sprinkle RH, Smith SJ, Buchanan GR. Intravenous narcotic therapy for children with severe sickle cell pain crisis. Am J Dis Child 1986;140:1255-1259.
12. Helvaci MR, Ayyildiz O, Gundogdu M. Hydroxyurea therapy and parameters of health in sickle cell patients. HealthMed 2014;8:451-456.
13. Leikin SL, Gallagher D, Kinney TR, Sloane D, Klug P, Rida W. Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatrics 1989; 84: 500-508.
14. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr 1985; 107: 861-866.
15. Sprinkle RH, Cole T, Smith S, Buchanan GR. Acute chest syndrome in children with sickle cell disease. A retrospective analysis of 100 hospitalized cases. Am J Pediatr Hematol Oncol 1986; 8: 105-110.
16. 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: 3107-3112.
17. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, 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 1995; 332: 1317-1322.
18. Charache S, Scott JC, Charache P. ‘‘Acute chest syndrome’’ in adults with sickle cell anemia. Microbiology, treatment, and prevention. Arch Intern Med 1979; 139: 67-69.
19. Davies SC, Luce PJ, Win AA, Riordan JF, Brozovic M. Acute chest syndrome in sickle-cell disease. Lancet 1984; 1: 36-38.
20. Helvaci MR, Aydin LY, Aydin Y. Chronic obstructive pulmonary disease may be one of the terminal end points of metabolic syndrome. Pak J Med Sci 2012;28:376-379.
21. Mannino DM, Watt G, Hole D, Gillis C, Hart C, McConnachie A, et al. The natural history of chronic obstructive pulmonary disease. Eur Respir J 2006;27:627-643.
22. Mapel DW, Hurley JS, Frost FJ, Petersen HV, Picchi MA, Coultas DB. Health care utilization in chronic obstructive pulmonary disease. A case-control study in a health maintenance organization. Arch Intern Med 2000; 160: 2653-2658.
23. Anthonisen NR, Connett JE, Enright PL, Manfreda J; Lung Health Study Research Group. Hospitalizations and mortality in the Lung Health Study. Am J Respir Crit Care Med 2002;166:333-339.
24. McGarvey LP, John M, Anderson JA, Zvarich M, Wise RA; TORCH Clinical Endpoint Committee. Ascertainment of cause-specific mortality in COPD: operations of the TORCH Clinical Endpoint Committee. Thorax 2007;62:411-415.
25. Kaminsky A, Sperling H. Diagnosis and management of priapism. Urologe A 2015; 54: 654-661.
26. Anele UA, Le BV, Resar LM, Burnett AL. How I treat priapism. Blood 2015; 125: 3551-3558.
27. Bartolucci P, Lionnet F. Chronic complications of sickle cell disease. Rev Prat 2014; 64: 1120-1126.
28. Broderick GA. Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy. J Sex Med 2012; 9: 88-103.
29. Ballas SK, Lyon D. Safety and efficacy of blood exchange transfusion for priapism complicating sickle cell disease. J Clin Apher 2015.
30. Xia M, Guerra N, Sukhova GK, Yang K, Miller CK, Shi GP, et al. Immune activation resulting from NKG2D/ligand interaction promotes atherosclerosis. Circulation 2011; 124: 2933-2943.
31. Mostafa A, Mohamed MK, Saeed M, Hasan A, Fontanet A, Godsland I, et al. Hepatitis C infection and clearance: impact on atherosclerosis and cardiometabolic risk factors. Gut 2010;59:1135-1140.
32. Bonora E, Targher G. Increased risk of cardiovascular disease and chronic kidney disease in NAFLD. Nat Rev Gastroenterol Hepatol 2012;9:372-381.
33. Anderson RN, Smith BL. Deaths: leading causes for 2001. Natl Vital Stat Rep 2003;52:1-85.
34. Helvaci MR, Kaya H. Effect of sickle cell diseases on height and weight. Pak J Med Sci 2011;27:361-364.
35. Helvaci MR, Sevinc A, Camci C, Keskin A. Atherosclerotic background of cirrhosis in sickle cell patients. Pren Med Argent 2014; 100: 127-133.
36. Levin A, Hemmelgarn B, Culleton B, Tobe S, McFarlane P, Ruzicka M, et al. Guidelines for the management of chronic kidney disease. CMAJ 2008; 179: 1154-1162.
37. Centers for Disease Control and Prevention (CDC). Prevalence of chronic kidney disease and associated risk factors--United States, 1999-2004. MMWR Morb Mortal Wkly Rep 2007; 56: 161-165.
38. Nassiri AA, Hakemi MS, Asadzadeh R, Faizei AM, Alatab S, Miri R, et al. Differences in cardiovascular disease risk factors associated with maximum and mean carotid intima-media thickness among hemodialysis patients. Iran J Kidney Dis 2012; 6: 203-208.
39. Helvaci MR, Aydin Y, Gundogdu M. Smoking induced atherosclerosis in cancers. HealthMED 2012; 6: 3744-3749.
40. Helvaci MR, Kaya H, Sevinc A, Camci C. Body weight and white coat hypertension. Pak J Med Sci 2009; 25: 916-921.
41. Tonelli M, Wiebe N, Culleton B, House A, Rabbat C, Fok M, et al. Chronic kidney disease and mortality risk: a systematic review. J Am Soc Nephrol 2006; 17: 2034-2047.
42. Helvaci MR, Aydin Y, Aydin LY. Atherosclerotic background of chronic kidney disease in sickle cell patients. HealthMED 2013; 7: 2532-2537.
43. Gueguen A, Mahevas M, Nzouakou R, Hosseini H, Habibi A, Bachir D, et al. Sickle-cell disease stroke throughout life: a retrospective study in an adult referral center. Am J Hematol 2014; 89: 267-272.
44. Majumdar S, Miller M, Khan M, Gordon C, Forsythe A, Smith MG, et al. Outcome of overt stroke in sickle cell anaemia, a single institution's experience. Br J Haematol 2014; 165: 707-713.
45. Helvaci MR, Aydogan F, Sevinc A, Camci C, Dilek I. Platelet and white blood cell counts in severity of sickle cell diseases. Pren Med Argent 2014; 100: 49-56.
46. Kossorotoff M, Grevent D, de Montalembert M. Cerebral vasculopathy in pediatric sickle-cell anemia. Arch Pediatr 2014;21:404-414.
47. Miller BA, Platt O, Hope S, Dover G, Nathan DG. Influence of hydroxyurea on fetal hemoglobin production in vitro. Blood 1987;70:1824-1829.
48. Charache S. Mechanism of action of hydroxyurea in the management of sickle cell anemia in adults. Semin Hematol 1997; 34: 15-21.
49. Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi CE, et al. NIH consensus development statement on hydroxyurea treatment for sickle cell disease. NIH Consens State Sci Statements 2008; 25: 1-30.
50. Tefferi A. Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management. Am J Hematol 2012; 87: 285-293.
51. Campion SN, Davenport SJ, Nowland WS, Cappon GD, Bowman CJ, Hurtt ME. Sensitive windows of skeletal development in rabbits determined by hydroxyurea exposure at different times throughout gestation. Birth Defects Res B Dev Reprod Toxicol 2012; 95: 238-249.
52. Charache S, Barton FB, Moore RD, Terrin ML, Steinberg MH, Dover GJ, et al. Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. The Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Medicine (Baltimore) 1996; 75: 300-326.
53. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA 2003; 289: 1645-1651.
54. Lebensburger JD, Miller ST, Howard TH, Casella JF, Brown RC, Lu M, et al. BABY HUG Investigators. Influence of severity of anemia on clinical findings in infants with sickle cell anemia: analyses from the BABY HUG study. Pediatr Blood Cancer 2012;59:675-678.