Hemolysis-associated priapism in sickle cell disease

Blood

Volumn 106, Issue 9, 2005, Pages 3264-3267

  Nolan, Vikki G ^b   Wyszynski, Diego F ^b   Farrer, Lindsay A ^b   Steinberg, Martin H ^a  

^a BOSTON MEDICAL CENTER   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ASPARTATE AMINOTRANSFERASE; BILIRUBIN; HEMOGLOBIN; LACTATE DEHYDROGENASE; NITRIC OXIDE;

ADOLESCENT; ADULT; ARTICLE; COMPARATIVE STUDY; CONTROLLED STUDY; DISEASE ASSOCIATION; HEMOLYSIS; HUMAN; LEUKOCYTE COUNT; MAJOR CLINICAL STUDY; MALE; PRIAPISM; PRIORITY JOURNAL; RETICULOCYTE COUNT; SICKLE CELL ANEMIA; STATISTICAL SIGNIFICANCE; THROMBOCYTE COUNT;

EID: 27644535417     PISSN: 00064971     EISSN: 00064971     Source Type: Journal    
DOI: 10.1182/blood-2005-04-1594     Document Type: Article

References (59)

1. Nagel R, Platt O. General pathophysiology of sickle cell anemia. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL eds. Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Cambridge, United Kingdom: Cambridge University Press; 2001.
2. Eland IA, van der Lei J, Stricker BH, Sturkenboom MJ. Incidence of priapism in the general population. Urology. 2001;57:970-972.
3. Diggs LW and Ching RE. Pathology of sickle cell anemia. Southern Med J. 1934;27:839-845.
4. Adedeji MO, Onuora VC, Ukoli FA. Haematological parameters associated with priapism in Nigerian patients with homozygous sickle cell disease. J Trop Med Hyg. 1988;91:157-159.
5. Al-Awamy B, Taha SA, Naeem MA. Priapism in association with sickle cell anemia in Saudi Arabia. Acta Haematol. 1985;73:181-182.
6. Badmus TA, Adediran IA, Adesunkanmi AR, Katung IA. Priapism in southwestern Nigeria. East Afr Med J. 2003;80:518-524.
7. Dahm P, Rao DS, Donatucci CF. Antiandrogens in the treatment of priapism. Urology. 2002;59:138.
8. Fowler JE Jr, Koshy M, Strub M, Chinn SK. Priapism associated with the sickle cell hemoglobinopathies: prevalence, natural history and sequelae. J Urol. 1991;145:65-68.
9. Gbadoe AD, Atakouma Y, Kusiaku K, Assimadi JK. Management of sickle cell priapism with etilefrine. Arch Dis Child. 2001;85:52-53.
10. Gbadoe AD, Dogba A, Segbena AY, et al. Priapism in sickle cell anemia in Togo: prevalence and knowledge of this complication. Hemoglobin. 2001;25:355-361.
11. Mantadakis E, Cavender JD, Rogers ZR, Ewalt DH, Buchanan GR. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 1999;21:518-522.
12. Okpala I, Westerdale N, Jegede T, Cheung B. Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol. 2002;118:918-921.
13. Saad ST, Lajolo C, Gilli S, et al. Follow-up of sickle cell disease patients with priapism treated by hydroxyurea. Am J Hematol. 2004;77:45-49.
14. Serjeant GR, de Ceulaer K, Maude GH. Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet. 1985;2:1274-1276.
15. Steinberg J, Eyre RC. Management of recurrent priapism with epinephrine self-injection and gonadotropin-releasing hormone analogue. J Urol. 1995;153:152-153.
16. Virag R, Bachir D, Lee K, Galacteros F. Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine. Urology. 1996;47:777-781.
17. Adeyoju AB, Olujohungbe AB, Morris J, et al. Priapism in sickle-cell disease; incidence, risk factors and complications: an international multicentre study. BJU Int. 2002;90:898-902.
18. Bruno D, Wigfall DR, Zimmerman SA, Rosoff PM, Wiener JS. Genitourinary complications of sickle cell disease. J Urol. 2001;166:803-811.
19. Gaston M, Rosse WF. The cooperative study of sickle cell disease: review of study design and objectives. Am J Pediatr Hematol Oncol. 1982;4:197-201.
20. Gaston M, Smith J, Gallagher D, et al. Recruitment in the Cooperative Study of Sickle Cell Disease (CSSCD). Control Clin Trials. 1987;8:131S-140S.
21. Management of sickle cell disease. Bethesda, MD: NIH, National Heart Lung and Blood Institute; revised May 28, 2002. NIH Publication No 02-2117 (4th ed).
22. de Ceulaer K, Higgs DR, Weatherall DJ, Hayes RJ, Serjeant BE, Serjeant GR. alpha-Thalassemia reduces the hemolytic rate in homozygous sickle-cell disease. N Engl J Med. 1983;309:189-190.
23. Embury SH, Clark MR, Monroy G, Mohandas N. Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes. J Clin Invest. 1984;73:116-123.
24. Embury SH, Dozy AM, Miller J, et al. Concurrent sickle-cell anemia and alpha-thalassemia: effect on severity of anemia. N Engl J Med. 1982;306:270-274.
25. Nagel R, Steinberg, MH. Hemoglobin SC disease and HbC disorders. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL, eds. Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Cambridge, United Kingdom: Cambridge University Press; 2001:756-785.
26. Reiter CD, Wang X, Tanus-Santos JE, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med. 2002;8:1383-1389.
27. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003;101:1257-1261.
28. 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. 2004;350:886-895.
29. Burnett AL. Role of nitric oxide in the physiology of erection. Biol Reprod. 1995;52:485-489.
30. Ohene-Frempong K, Steinberg MH. Clinical aspects of sickle cell anemia in adults and children. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL, eds. Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Cambridge, United Kingdom: Cambridge University Press; 2001:611-670.
31. Burnett AL. Priapism pathophysiology: clues to prevention. Int J Impot Res. 2003;15(suppl 5):S80-85.
32. Burnett AL. Pathophysiology of priapism: dysregulatory erection physiology thesis. J Urol. 2003;170:26-34.
33. Champion HC, Bivalacqua TJ, Takimoto E, Kass DA, Burnett AL. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A. 2005;102:1661-1666.
34. Adams RJ, Kutlar A, McKie V, et al. Alpha thalassemia and stroke risk in sickle cell anemia. Am J Hematol. 1994;45:279-282.
35. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288-294.
36. Powars DR, Weiss JN, Chan LS, Schroeder WA. Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia? Blood. 1984;63:921-926.
37. Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994;84:643-649.
38. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med. 1991;325:11-16.
39. Sebastiani P, Ramoni MF, Nolan V, Baldwin CT, Steinberg MH. Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia. Nat Genet. 2005;37:435-440.
40. Steinberg MH, Embury SH. Alpha-thalassemia in blacks: genetic and clinical aspects and interactions with the sickle hemoglobin gene. Blood. 1986;68:985-990.
41. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325:1476-1481.
42. Milner PF, Kraus AP, Sebes JI, et al. Osteonecrosis of the humeral head in sickle cell disease. Clin Orthop Relat Res. 1993;289:136-143.
43. Newburger PE, Subrahmanyam YV, Weissman SM. Global analysis of neutrophil gene expression. Curr Opin Hematol. 2000;7:16-20.
44. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-1644.
45. Fadlon E, Vordermeier S, Pearson TC, et al. Blood polymorphonuclear leukocytes from the majority of sickle cell patients in the crisis phase of the disease show enhanced adhesion to vascular endothelium and increased expression of CD64. Blood. 1998;91:266-274.
46. Matsui NM, Borsig L, Rosen SD, et al. P-selectin mediates the adhesion of sickle erythrocytes to the endothelium. Blood. 2001;98:1955-1962.
47. Udani M, Zen Q, Cottman M, et al. Basal cell adhesion molecule Lutheran protein: the receptor critical for sickle cell adhesion to laminin. J Clin Invest. 1998;101:2550-2558.
48. Hebbel RP. Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest. 1997;99:2561-2564.
49. 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. 1998;339:5-11.
50. Morris CR, Morris SM Jr, Hagar W, et al. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med. 2003;168:63-69.
51. Chockalingam A, Gnanavelu G, Venkatesan S, et al. Efficacy and optimal dose of sildenafil in primary pulmonary hypertension. Int J Cardiol. 2005;99:91-95.
52. Derchi G, Forni GL, Formisano F, et al. Efficacy and safety of sildenafil in the treatment of severe pulmonary hypertension in patients with hemoglobinopathies. Haematologica. 2005;90:452-458.
53. Ware RE, Zimmerman SA, Schultz WH. Hydroxyurea as an alternative to blood transfusions for the prevention of recurrent stroke in children with sickle cell disease. Blood. 1999;94:3022-3026.
54. Ballas SK, Marcolina MJ. Determinants of red cell survival and erythropoietic activity in patients with sickle cell anemia in the steady state. Hemoglobin. 2000;24:277-286.
55. Cokic VP, Smith RD, Beleslin-Cokic BB, et al. Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest. 2003;111:231-239.
56. Pacelli R, Taira J, Cook JA, Wink DA, Krishna MC. Hydroxyurea reacts with heme proteins to generate nitric oxide [letter]. Lancet. 1996;347:900.
57. Nolan VG, Baldwin C, Ma Q, et al. Association of single nucleotide polymorphisms in klotho with priapism in sickle cell anaemia. Br J Haematol. 2005;128:266-272.
58. Saito Y, Yamagishi T, Nakamura T, et al. Klotho protein protects against endothelial dysfunction. Biochem Biophys Res Commun. 1998;248:324-329.
59. Bialecki ES, Bridges KR. Sildenafil relieves priapism in patients with sickle cell disease [letter]. Am J Med. 2002;113:252.