Thrombotic thrombocytopenic purpura epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2] Anum Ijaz M.B.B.S., M.D.[3]
Overview
The most proportion of TTP cases occurred after 40 years, black race and femal sex. Congenital forms occur in children.
Epidemiology and Demographics
Incidence
- Thrombotic thrombocytopenic purpura (TTP) is a rare disorder with a worldwide annual incidence of approximately 2–6 cases per million population.[1],[2],[3]
- Data suggests the increased risk of TTP in black race and femal sex.[4]
- In adults, more than 95% of TTP cases are immune-mediated (iTTP), while only 3%–5% represent congenital ADAMTS13 deficiency.[1]
Prevalence
- In 2005, the prevalence of TTP was estimated to be 3 cases per 1000,000 individuals worldwide.[5]
Age
- Patients of all age groups may developTTP.
- The incidence of TTP increases with age; the median age at diagnosis is 40 years. TTP often occurs after 40 years.
- The incidence of immune TTP is substantially higher in adults than in children, with an incident rate ratio (IRR) of 31.62 per 100 000 person-years.[2]
Race
- iTTP disproportionately affects Black individuals, who have an incident rate ratio of 7.09 per 100,000 years as compared with non-Black populations.[2]
Gender
- Females are more commonly affected by iTTP than men.
- The females to men ratio is approximately 2 to 1.[6][2] and the incident rate ratio is 3.19 per 100,000 person-years as compared to males.
Region
- The majority of TTP cases are reported in black african and caribbean people.[7]
References
- ↑ 1.0 1.1 Mariotte E, Azoulay E, Galicier L, Rondeau E, Zouiti F, Boisseau P, Poullin P, de Maistre E, Provôt F, Delmas Y, Perez P, Benhamou Y, Stepanian A, Coppo P, Veyradier A (May 2016). "Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy". Lancet Haematol. 3 (5): e237–45. doi:10.1016/S2352-3026(16)30018-7. PMID 27132698.
- ↑ 2.0 2.1 2.2 2.3 Reese JA, Muthurajah DS, Kremer Hovinga JA, Vesely SK, Terrell DR, George JN (October 2013). "Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features". Pediatr Blood Cancer. 60 (10): 1676–82. doi:10.1002/pbc.24612. PMID 23729372.
- ↑ Miesbach W, Menne J, Bommer M, Schönermarck U, Feldkamp T, Nitschke M, Westhoff TH, Seibert FS, Woitas R, Sousa R, Wolf M, Walzer S, Schwander B (November 2019). "Incidence of acquired thrombotic thrombocytopenic purpura in Germany: a hospital level study". Orphanet J Rare Dis. 14 (1): 260. doi:10.1186/s13023-019-1240-0. PMC 6858672 Check
|pmc=value (help). PMID 31730475. - ↑ Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN (November 2010). "Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes". Am. J. Hematol. 85 (11): 844–7. doi:10.1002/ajh.21833. PMC 3420337. PMID 20799358.
- ↑ Terrell DR, Williams LA, Vesely SK, Lämmle B, Hovinga JA, George JN (July 2005). "The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency". J. Thromb. Haemost. 3 (7): 1432–6. doi:10.1111/j.1538-7836.2005.01436.x. PMID 15978100.
- ↑ Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN (November 2010). "Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes". Am. J. Hematol. 85 (11): 844–7. doi:10.1002/ajh.21833. PMC 3420337. PMID 20799358.
- ↑ Martino S, Jamme M, Deligny C, Busson M, Loiseau P, Azoulay E, Galicier L, Pène F, Provôt F, Dossier A, Saheb S, Veyradier A, Coppo P (2016). "Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors". PLoS ONE. 11 (7): e0156679. doi:10.1371/journal.pone.0156679. PMC 4934773. PMID 27383202.
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