Thrombotic thrombocytopenic purpura - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Thrombotic thrombocytopenic purpura (TTP) is a potential diagnosis in any patient with hemolytic anemia and thrombocytopenia - 95% of cases are fatal if left untreated.
Symptoms are usually nonspecific, although half of patients have neurologic abnormalities. Pentad of fever, renal failure, hemolytic anemia, thrombocytopenia, and neurologic changes are often seen, although most patients do not have the entire pentad.
Examination of the peripheral smear is critical and shows evidence of microangiopathic hemolytic anemia with fragmented red blood cells (schistocytes) and thrombocytopenia.
An urgent hematology consultation is recommended for suspected cases.
Plasma-exchange therapy combined with corticosteroids is the mainstay of treatment for acute acquired (idiopathic) TTP. Caplacizumab may be prescribed as an adjunctive therapy in adults.

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History & Exam
- Key Factors
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Peripheral smear of a patient with TTP showing many fragmented, partly rounded cells. Also note the lack of platelets

Citations

Key Articles

Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017 Feb;15(2):312-22.[Abstract][Full Text]
Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35.[Abstract][Full Text]
Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005 Jul;3(7):1432-6.[Abstract][Full Text]
Moschowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89.
Moore JC, Hayward CP, Warkentin TE, et al. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98:1842-1846.[Abstract][Full Text]
Banno F, Kokame K, Okuda T, et al. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006 Apr 15;107(8):3161-6.[Abstract][Full Text]
Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost. 2005 Nov;3(11):2420-7.[Abstract]
Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991 Aug 8;325(6):393-7.[Abstract]
Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021 Apr 8;137(14):1855-61.[Abstract][Full Text]

Referenced Articles

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2. Vesely SK, George JN, Lammle B, et al. ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003;102:60-68.[Abstract][Full Text]
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24. Moore JC, Hayward CP, Warkentin TE, et al. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98:1842-1846.[Abstract][Full Text]
25. Banno F, Kokame K, Okuda T, et al. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006 Apr 15;107(8):3161-6.[Abstract][Full Text]
26. Crawley JT, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program. 2013;2013:292-9.[Abstract]
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44. Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost. 2005 Nov;3(11):2420-7.[Abstract]
45. Shih AW, McFarlane A, Verhovsek M. Haptoglobin testing in hemolysis: measurement and interpretation. Am J Hematol. 2014 Apr;89(4):443-7.[Abstract]
46. Vesely SK, George JN, Lammle B, et al. ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003 Jul 1;102(1):60-8.[Abstract][Full Text]
47. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009 Oct 22;361(17):1676-87.[Abstract]
48. Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006 Aug 15;108(4):1267-79.[Abstract][Full Text]
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61. Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021 Apr 8;137(14):1855-61.[Abstract][Full Text]
62. Swisher KK, Terrell DR, Vesely SK, et al. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion. 2009 May;49(5):873-87.[Abstract]
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