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Questions and Answers
Which factor has the shortest half-life among the vitamin K-dependent factors?
Which factor has the shortest half-life among the vitamin K-dependent factors?
What is the most common cause of vitamin K deficiency?
What is the most common cause of vitamin K deficiency?
Which patients may require smaller than expected doses of warfarin therapy?
Which patients may require smaller than expected doses of warfarin therapy?
What is the treatment of choice for vitamin K deficiency?
What is the treatment of choice for vitamin K deficiency?
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Which treatment administration route should be avoided in patients with coagulopathies to prevent hematomas?
Which treatment administration route should be avoided in patients with coagulopathies to prevent hematomas?
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What is the mainstay of Thrombotic Thrombocytopenic Purpura (TTP) treatment?
What is the mainstay of Thrombotic Thrombocytopenic Purpura (TTP) treatment?
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Which enzyme cleaves von Willebrand factor multimers in the context of TTP?
Which enzyme cleaves von Willebrand factor multimers in the context of TTP?
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What establishes the diagnosis of TTP?
What establishes the diagnosis of TTP?
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Which manifestations are classic in patients with TTP?
Which manifestations are classic in patients with TTP?
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What is the role of immunosuppressive drugs like prednisone or rituximab in TTP treatment?
What is the role of immunosuppressive drugs like prednisone or rituximab in TTP treatment?
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What is the effect of Vitamin K administration in TTP patients?
What is the effect of Vitamin K administration in TTP patients?
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What predicts the likelihood of finding low ADAMTS13 activity diagnostic for TTP?
What predicts the likelihood of finding low ADAMTS13 activity diagnostic for TTP?
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What is the recommended route for administering TTP treatment?
What is the recommended route for administering TTP treatment?
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What may be present in addition to thrombocytopenia and microangiopathic hemolytic anemia in TTP diagnosis?
What may be present in addition to thrombocytopenia and microangiopathic hemolytic anemia in TTP diagnosis?
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What is the effect of reduced ADAMTS13 activity in TTP?
What is the effect of reduced ADAMTS13 activity in TTP?
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When should Thrombotic Thrombocytopenic Purpura (TTP) be treated?
When should Thrombotic Thrombocytopenic Purpura (TTP) be treated?
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What is the primary cause of coagulopathy in liver disease?
What is the primary cause of coagulopathy in liver disease?
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Which test is used for screening coagulation factor deficiencies in liver disease?
Which test is used for screening coagulation factor deficiencies in liver disease?
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What is the prevalence of Idiopathic Thrombocytopenia Purpura (ITP)?
What is the prevalence of Idiopathic Thrombocytopenia Purpura (ITP)?
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What is the recommended diagnostic approach for ITP?
What is the recommended diagnostic approach for ITP?
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What is the primary treatment for liver disease-induced coagulopathy?
What is the primary treatment for liver disease-induced coagulopathy?
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What may cause secondary ITP?
What may cause secondary ITP?
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What is the gender primarily affected by ITP?
What is the gender primarily affected by ITP?
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What is the most reliable test for diagnosing ITP?
What is the most reliable test for diagnosing ITP?
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What is the association of severe liver disease with thrombosis?
What is the association of severe liver disease with thrombosis?
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What is the effect of liver disease on PT and aPTT?
What is the effect of liver disease on PT and aPTT?
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Which factor is particularly affected by impaired production in liver disease-induced coagulopathy?
Which factor is particularly affected by impaired production in liver disease-induced coagulopathy?
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What is the primary cause of coagulopathy in liver disease?
What is the primary cause of coagulopathy in liver disease?
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What is the primary cause of coagulopathy in liver disease?
What is the primary cause of coagulopathy in liver disease?
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Which test is disproportionately longer in patients with liver disease-induced coagulopathy?
Which test is disproportionately longer in patients with liver disease-induced coagulopathy?
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What is the prevalence of Idiopathic Thrombocytopenia Purpura (ITP)?
What is the prevalence of Idiopathic Thrombocytopenia Purpura (ITP)?
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What is the recommended treatment for liver disease-induced coagulopathy?
What is the recommended treatment for liver disease-induced coagulopathy?
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What is the primary age group affected by Idiopathic Thrombocytopenia Purpura (ITP)?
What is the primary age group affected by Idiopathic Thrombocytopenia Purpura (ITP)?
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What is the main screening test for coagulation factor deficiencies in liver disease?
What is the main screening test for coagulation factor deficiencies in liver disease?
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What is the most reliable diagnostic evidence for Idiopathic Thrombocytopenia Purpura (ITP)?
What is the most reliable diagnostic evidence for Idiopathic Thrombocytopenia Purpura (ITP)?
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What is the effect of severe liver disease on fibrinolysis and thrombosis?
What is the effect of severe liver disease on fibrinolysis and thrombosis?
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What is the primary factor affected in liver disease-induced coagulopathy?
What is the primary factor affected in liver disease-induced coagulopathy?
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In which patients is coagulopathy primarily seen in liver disease?
In which patients is coagulopathy primarily seen in liver disease?
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What is the recommended treatment for severe liver disease-induced coagulopathy?
What is the recommended treatment for severe liver disease-induced coagulopathy?
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When is a bone marrow examination required for diagnosing ITP?
When is a bone marrow examination required for diagnosing ITP?
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What is the scoring system for Heparin-induced thrombocytopenia (HIT)?
What is the scoring system for Heparin-induced thrombocytopenia (HIT)?
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What percentage of patients with low pretest probability of HIT have HIT antibodies?
What percentage of patients with low pretest probability of HIT have HIT antibodies?
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What should be done if HIT is suspected?
What should be done if HIT is suspected?
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What is the mainstay of treatment for Thrombotic Thrombocytopenic Purpura (TTP)?
What is the mainstay of treatment for Thrombotic Thrombocytopenic Purpura (TTP)?
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What are the conditions that can cause Disseminated Intravascular Coagulation (DIC)?
What are the conditions that can cause Disseminated Intravascular Coagulation (DIC)?
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What are the characteristics of acute DIC?
What are the characteristics of acute DIC?
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What do D-dimer and fibrin degradation products reflect in DIC?
What do D-dimer and fibrin degradation products reflect in DIC?
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What should testing for DIC include?
What should testing for DIC include?
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What is the recommended duration of anticoagulation for HIT?
What is the recommended duration of anticoagulation for HIT?
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When can patients with a history of HIT be safely reexposed to heparin?
When can patients with a history of HIT be safely reexposed to heparin?
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What is the recommended initial treatment for all patients with thrombocytopenia?
What is the recommended initial treatment for all patients with thrombocytopenia?
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In refractory cases of thrombocytopenia, which of the following immunosuppressants may be used?
In refractory cases of thrombocytopenia, which of the following immunosuppressants may be used?
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What is the primary cause of Heparin-Induced Thrombocytopenia (HIT)?
What is the primary cause of Heparin-Induced Thrombocytopenia (HIT)?
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When does HIT typically manifest after starting any kind of heparin?
When does HIT typically manifest after starting any kind of heparin?
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What percentage of patients who have HIT develop thrombosis?
What percentage of patients who have HIT develop thrombosis?
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What is the most sensitive, readily available screening test for HIT?
What is the most sensitive, readily available screening test for HIT?
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What does a lower platelet count than usual suggest in HIT?
What does a lower platelet count than usual suggest in HIT?
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What is the primary treatment for all patients with thrombocytopenia?
What is the primary treatment for all patients with thrombocytopenia?
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What may be used for treatment of refractory cases of thrombocytopenia?
What may be used for treatment of refractory cases of thrombocytopenia?
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Study Notes
Thrombotic Thrombocytopenic Purpura (TTP) - Key Points
- Parenteral treatment should be administered subcutaneously or intravenously, not intramuscularly
- Intramuscular injections should be avoided in patients with coagulopathies to prevent hematomas
- Vitamin K administration takes 18–24 hours to have effect; fresh frozen plasma or prothrombin complex concentrate may be required for bleeding patients
- Patients with TTP exhibit systemic illness with classic manifestations including thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, fever, and acute kidney injury
- TTP diagnosis requires thrombocytopenia and microangiopathic hemolytic anemia; neurologic abnormalities and acute kidney injury may also be present
- ADAMTS13 enzyme cleaves von Willebrand factor multimers; its deficiency leads to platelet aggregation and microcirculation clumping
- Reduced ADAMTS13 activity and positive anti-ADAMTS13 antibody test establish the diagnosis of TTP
- The "PLASMIC" score predicts the likelihood of finding low ADAMTS13 activity diagnostic for TTP
- Plasma exchange is the mainstay of TTP treatment, improving survival rates from 90% mortality to 90% survival
- Immunosuppressive drugs like prednisone or rituximab are used to reduce the production of anti-ADAMTS13 antibody
- Thrombotic Thrombocytopenic Purpura (TTP) should be treated when suspected, and a definitive diagnosis is not necessary to initiate treatment
- Liver disease-induced coagulopathy may present asymptomatically or incidentally on coagulation laboratory studies
Heparin-Induced Thrombocytopenia and Disseminated Intravascular Coagulation
- Heparin-induced thrombocytopenia (HIT) scoring system: 0-3 points indicate low probability, 4-5 points indicate intermediate probability, and 6-8 points indicate high probability.
- HIT antibodies were found in 0.9% of patients with low pretest probability, 11.4% in those with intermediate scores, and 34% in those with high scores.
- Heparin must be discontinued if HIT is suspected, and an alternative anticoagulant such as argatroban or fondaparinux should be started.
- Low-molecular-weight heparin should not be substituted, and warfarin should not be used until platelet count recovers.
- Anticoagulation should continue for 2-3 months.
- Patients with a history of HIT can be safely reexposed to heparin after a year, if necessary.
- Disseminated Intravascular Coagulation (DIC) presents as uncontrolled spontaneous diffuse bleeding in patients with severe illness.
- Conditions that cause DIC include trauma, advanced adenocarcinomas, obstetric crises, and acute promyelocytic leukemia.
- In some cases of DIC, clotting manifestations may predominate, leading to recurrent thrombosis without bleeding.
- Acute DIC is characterized by thrombocytopenia, prolongation of PT/INR and aPTT, reduction of plasma fibrinogen level, and increases in D-dimer and fibrin degradation products.
- D-dimer and FDP reflect fibrinolytic activity, and fibrinogen levels below 100 mg/dL may correlate with bleeding risk in DIC.
- Testing for DIC should include platelet count, PT/INR, aPTT, fibrinogen, and D-dimer.
Treatment of Thrombocytopenia and Heparin-Induced Thrombocytopenia
- Serologic studies are indicated if SLE, hepatitis C, or HIV infection is suspected.
- High-dose corticosteroid, such as prednisone or dexamethasone, is the initial treatment for all patients with thrombocytopenia.
- Patients who do not respond to corticosteroids or whose thrombocytopenia recurs when the corticosteroids are stopped may undergo splenectomy.
- In refractory cases of thrombocytopenia, other immunosuppressants may be used, such as rituximab, azathioprine, or cyclophosphamide.
- Thrombopoietin analogues such as romiplostim and eltrombopag have become more widely used for treatment of refractory cases of thrombocytopenia.
- Heparin-Induced Thrombocytopenia (HIT) is caused by the development of an antibody directed against a heparin-platelet factor 4 complex.
- HIT develops in about 5% of patients who receive heparin, with surgical patients at higher risk.
- HIT manifests between 5 and 10 days after starting any kind of heparin.
- Thrombosis develops in about 50% of patients who have HIT.
- The most sensitive, readily available screening test for HIT is an enzyme-linked immunosorbent assay (ELISA) for anti-PF4 antibody.
- A pretest probability scoring system (the 4“T’s”) has been validated for the diagnosis of HIT, considering thrombocytopenia, timing of platelet fall, and thrombosis or other sequelae.
- The platelet count does not usually drop below 50,000/mcL in HIT; a lower platelet count suggests another etiology.
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Description
Test your knowledge of Thrombotic Thrombocytopenic Purpura (TTP) and Heparin-Induced Thrombocytopenia and Disseminated Intravascular Coagulation (DIC) with this comprehensive quiz. Review key points on diagnosis, treatment, and management of these critical hematologic disorders.
