       Document 0057
 DOCN  M9610057
 TI    Increasing mortality from thrombotic thrombocytopenic purpura in the
       United States--analysis of national mortality data, 1968-1991.
 DT    9601
 AU    Torok TJ; Holman RC; Chorba TL; Division of Viral and Rickettsial
       Diseases, Centers for Disease; Control and Prevention, Atlanta, Georgia
       30333, USA.
 SO    Am J Hematol. 1995 Oct;50(2):84-90. Unique Identifier : AIDSLINE
       MED/96016354
 AB    Thrombotic thrombocytopenic purpura (TTP) is a rare disease and the
       epidemiologic features have been incompletely characterized. Because of
       the historically high case-fatality rate for TTP, we analyzed U.S.
       multiple cause-of-death mortality data with TTP listed on the death
       record for the period 1968-1991, in order to estimate the incidence of
       TTP, to characterize demographic features of the decedents, and to
       determine if trends in mortality correlate with findings from clinical
       studies showing improved survival in recent years. There were 4,523
       TTP-associated deaths during the 24-year study period. The annual
       age-adjusted mortality rate decreased initially and reached its lowest
       point at 0.4 per 1,000,000 residents for the years 1970 through 1973,
       and then increased steadily to 1.1 during the last 4 years of the study
       period, 1988 through 1991. We estimate the current incidence of TTP to
       be approximately 3.7 cases per 1,000,000 residents. Deaths were rare
       below the age of 20 years, but the age-specific mortality rate for those
       20 years and older increased steadily with increasing age. Regardless of
       age, females were affected more often than males, and the overall
       female-to-male age-adjusted rate ratio was 1.9 (95% confidence interval
       (CI), 1.8 to 2.0). The greatest age-specific difference was between
       females and males in their twenties (rate ratio 3.2; 95% CI, 2.6 to
       3.9). The mortality rate for blacks, and especially black females, was
       higher than the mortality rate for whites (black-to-white age-adjusted
       rate ratio 3.4; 95% CI, 3.2 to 3.6; black female-to-white female
       age-adjusted rate ratio 3.6; 95% CI, 3.3 to 3.9), although the majority
       of deaths were among whites (71.5%). Infection with the human
       immunodeficiency virus (HIV) or an HIV-related diagnosis was reported in
       61 (1.3%) decedents overall and in 51 (4.4%) decedents from 1988 through
       1991. The TTP mortality rate has increased over time despite reports of
       significant improvement in survival associated with clinical use of
       plasma infusion and plasma exchange. This trend in mortality suggests
       that the incidence of TTP is increasing. Blacks, and black females in
       particular, are affected at a disproportionately high rate. The
       increased incidence of HIV infection and related disease may have
       contributed to some of the increase in TTP mortality in recent years,
       but it does not explain the majority of the increase, which began before
       the onset of the HIV epidemic.
 DE    Adolescence  Adult  Aged  Aged, 80 and over  Caucasoid Race  Child
       Child, Preschool  Comparative Study  Female  Human  HIV
       Infections/COMPLICATIONS  Infant  Male  Middle Age  Negroid Race
       Purpura, Thrombotic Thrombocytopenic/COMPLICATIONS/*MORTALITY  Sex
       Characteristics  United States  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

