Sudden Cardiac Death From Thrombotic Thrombocytopenic Purpura

Abstract

Clinical thrombotic thrombocytopenic purpura (TTP) is characterized by a pentad of microangiopathic hemo lytic anemia, thrombocytopenia, neurological symptoms, renal involvement, and fever. A case of TTP in which early symp toms and signs were suggestive of ischemic heart disease, renal failure, and severe thrombocytopenia developed to a rapid out come of death. The postmortem examination revealed coronary artery microthrombi, typical of TTP. The clinical presentation of this TTP was atypical: severe thrombocytopenia, striking renal and CNS symptoms were present, but fever and anemia were not present. Thrombotic thrombocytopenic purpura is an uncommon condition that carries a high fatality rate if un treated. Awareness of this syndrome and its high risk of sudden death underlines the importance of rapid diagnosis and treat ment.

Keywords

Key Words: Thrombotic thrombocytopenic purpura Atypical presentation Sudden cardiac death Coronary ar tery microthrombi

References

Bick RL Quanlitative platelet defects. In Hematology: Clinical and Laboratory Practice. Bick RL , Bennet JM , Brynes RK et al. (eds.). St. Lewis, MO: Mosby; 1993:1347.

Webb JG , Butany J. , Langer G. , Scott JG , Lin PP Myocardites and myocardial hemorrhage associated with thrombotic thrombocytopenic purpura. Arch Intern Med 1990;150:1535.

Bell MD, Barnhart JSJr, Martin J.M. Thrombotic thrombocytopenic purpura causing sudden, unexpected death—a series of eight patients . J Forensic Sci 1990;35:601.

Pannarale G. , Vicchio D. , Gattini G. , Romeo F. , Reale A. Cardiac involvement in a case of thrombotic thrombocytopenic purpura. Cardiologia 1986 ;31:163.

Siersema PD , Kras JM , Van den Bery B. Cardiac manifestations of thrombotic thrombocytopenic purpura. Neth J Med 1989;35:100.

Ridolfi R. , Hutchins G. , Bell W. The heart and cardiac conduction system in thrombotic thrombocytopenic purpura . Ann Intern Med 1979;81:257.