Thrombotic Thrombocytopenic Purpura
Basics
Description
Description
- Thrombotic thrombocytopenic purpura (TTP) is a severe disorder of abnormal clotting affecting multiple organ systems
- Classically characterized by pentad of:
- Thrombocytopenia
- Hemolytic anemia
- Mild renal dysfunction
- Neurologic signs
- Fever
- Uncommon to see all 5 features in a single patient; if present, severe end-organ damage or ischemia has likely taken place
- Thrombocytopenia and hemolytic anemia are the most common features
- Associated with acquired or congenital deficiency of plasma von Willebrand factor–cleaving protease (ADAMTS13)
- Acquired TTP – primary thrombotic microangiopathy with severe ADAMTS13 deficiency
- Hereditary TTP – inherited thrombotic microangiopathy with inherited ADAMTS13 mutations
Classic Course
- Acute onset
- Fulminant course lasting days to a few months
- Nearly always fatal without treatment:
- >90% mortality without treatment
- Reverses to >90% survival with modern treatment
- Clinical presentations include:
- Hereditary or acquired
- Familial, chronic, or relapsing
- Drug induced:
- Allergic or immune mediated (quinine, ticlopidine, clopidogrel)
- Dose-related toxicity (mitomycin C, cyclosporine)
- Pregnancy, postpartum associated:
- 10–25% of cases
- Bone marrow transplantation associated
- Infection
- Acquired TTP incidence is approximately three cases per one million adults per year
- Median age is 41. Range is 9–78
- Acquired TTP is uncommon in pediatric or geriatric populations
- Women affected about twice as frequently as men
- More common in African Americans
Etiology
Etiology
- Unknown primary stimulant; possibly systemic endothelial cell damage results inactivation of coagulation pathway
- May also be due to other autoimmune disorders such as lupus
- Platelet aggregation and fibrin deposition occurring in arterioles and capillaries leading to microthrombi and obstruction to blood flow
- Platelet aggregation leads to:
- Consumption of platelets
- Widespread microvascular hyaline thrombotic lesions
- Microvasculature obstruction with platelet aggregates leads to:
- Red cell hemolysis
- Accumulation of heme breakdown products
- Anemia
- End-organ ischemia results from diffuse thrombosis in small vessels:
- Most common in heart, brain, kidney, pancreas, and adrenal glands
- Deficiency of vWFcp causes failure of control of coagulation pathway
Risk Factors
GeneticsRisk Factors
- Some cases are genetic/familial
- VWFcp was recently identified as new member of ADAMTS family and designated ADAMTS13
- Mutations in ADAMTS13 gene cause autosomal recessive form of chronic relapsing TTP
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Citation
Schaider, Jeffrey J., et al., editors. "Thrombotic Thrombocytopenic Purpura." 5-Minute Emergency Consult, 6th ed., Lippincott Williams & Wilkins, 2020. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307542/all/Thrombotic_Thrombocytopenic_Purpura.
Thrombotic Thrombocytopenic Purpura. In: Schaider JJJ, Barkin RMR, Hayden SRS, et al, eds. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307542/all/Thrombotic_Thrombocytopenic_Purpura. Accessed November 8, 2024.
Thrombotic Thrombocytopenic Purpura. (2020). In Schaider, J. J., Barkin, R. M., Hayden, S. R., Wolfe, R. E., Barkin, A. Z., Shayne, P., & Rosen, P. (Eds.), 5-Minute Emergency Consult (6th ed.). Lippincott Williams & Wilkins. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307542/all/Thrombotic_Thrombocytopenic_Purpura
Thrombotic Thrombocytopenic Purpura [Internet]. In: Schaider JJJ, Barkin RMR, Hayden SRS, Wolfe RER, Barkin AZA, Shayne PP, Rosen PP, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. [cited 2024 November 08]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307542/all/Thrombotic_Thrombocytopenic_Purpura.
* Article titles in AMA citation format should be in sentence-case
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BT - 5-Minute Emergency Consult
UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307542/all/Thrombotic_Thrombocytopenic_Purpura
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DB - Emergency Central
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