We present a rare case of unexpected sudden death in a young woman with undiagnosed sickle disease. The provocative factors for the terminal events were excessive exercise in the form of trekking, urinary infection, and emotional stress the sudden cardiovascular collapse could have resulted from acute pulmonary hypertension resulting from severe hemolysis or acute chest syndrome.
Kar B, Satapathy RK, Kulozik AE, Kulozik M, Sir S, Serjeant BE, et al. Sickle Cell Disease in Orissa state, India; Lancet 1986;2:1198-201.
Edward J, Benz Jr. Haemoglobinopathies. In: Braunwald, Fauci, Kasper, Hauser, Longo, Jameson, editors. Harrison′s Principles of Internal Medicine. 15Th edn. Mcgrath Hill:669-70.
Steinberg MH, Embury SH. Alpha-thalassemia in blacks:genetic and clinical aspects and interactions with the sickle hemoglobin gene. Blood 1986;68:985.
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;330:1639-44.
Errol L. Fields. Phenotypic variation in sickle cell disease: An Analysis. www.sickle.bwn.harvard.edu/sickle_heterogeniety. html.
Gladwin MT, Sachdev V, Jison ML. Pulmonary hypertension as a risk factor for death in patients with Sickle Cell Diseases. N Engl J Med 2004;350;9:886-95.
Noguchi CT, Rodgers GP, Serjeant G, Schechter AN. Levels of foetal hemoglobin necessary for treatement of sickle cell disease. N Engl J Med 1998;318:96-9.
Seltzer WK, Abshire TC, Lane PA, Roloff JS, Githens, JH. Molecular genetic studies in black families with sickle cell anemia and unusually high levels of fetal hemoglobin. Hemoglobin 1992;16:363-77.
Davies SC, Luce PJ, Win AA, Riorden JP. Acute chest syndrome in sickle cell. Lancet 1984;1:36-8.
Quin CT, Buchanan GR, Acute chest syndrome of sickle cell disease. Journal of paediatrics October 1994;135.
Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, et al. Acute chest syndrome in sickle cell disease, incidence and risk factors. The cooperative study of sickle cell disease. Blood 1994;84:643-9.
Covitz W, Espeland M, Gallager D, Hellenbrand W, The heart in sickle cell anaemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest 1995;108:1214-9.
Romero Mestre JC, Hernandez A, Agramonte O, Hernandez P. Cardiovascular autonomic dysfunction in sickle cell anemia. A possible risk factor for death ? Clin. Auton Res 1997;7:1-5.
Elliott P. Vichinsky. Pulmonary hypertension in sickle cell disease. N Engl J Med 2004;350;9:857-9.
Powers D, Weidman J A, Odom - Maryon T, Wiland J C, Johnson CS. Sickle cell chronic lung disease:prior morbidity and the risk of pulmonary failure. Medicine 1988;67:66-76.
Reiter CD, Wang X, Tanus-Santos JE. Cell-free hemoglobin limits nitric oxide bio availability in sickle cell disease. Nat Med 2002;8:1389-9.
Kark J A, Posey DM, Schumacher HR, Ruehle CJ. Sickle cell trait as a risk factor for sudden death in physical training. N Engl J Med 1987;317:781-7.