Thrombotic Thrombocytopenic Purpura Treatment Strategies
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Questions and Answers

What is inferred when a patient presents with bleeding but no anatomical cause can be discovered?

  • Primary coagulation factor deficiency
  • Functional impairment of the normal hemostatic process (correct)
  • Excessive platelet activation
  • Structural abnormalities in blood vessels

Which disorder is characterized by frequent bleeding episodes primarily from the nose and gastrointestinal tract?

  • Hereditary hemorrhagic telangiectasia (correct)
  • Steroid purpura
  • Henoch-Schonlein syndrome
  • Ehlers-Danlos syndrome

What is the typical presentation of hemarthrosis?

  • Bleeding in the joints (correct)
  • Petechiae all over the body
  • Intracranial hemorrhage
  • Characteristic skin bruising

Which condition is associated with abnormal telangiectatic capillaries resulting in bleeding episodes?

<p>Hereditary hemorrhagic telangiectasia (A)</p> Signup and view all the answers

In what percentage of cases do individuals with hereditary hemorrhagic telangiectasia develop pulmonary AVM?

<p>40% (D)</p> Signup and view all the answers

At what age, on average, does epistaxis (nosebleeds) begin in individuals with hereditary hemorrhagic telangiectasia?

<p>&gt;12 years old (B)</p> Signup and view all the answers

Which condition is characterized by the need for vitamin C to synthesize hydroxyproline, an essential constituent of collagen?

<p>Scurvy (B)</p> Signup and view all the answers

Which condition is associated with (+) acute inflammatory reaction with IgA and complement components in capillaries, mesangial tissues, and small arterioles?

<p>Henoch-Schönlein purpura (B)</p> Signup and view all the answers

In which condition can glucocorticoid therapy lead to skin bleeding and easy bruising due to atrophy of supporting connective tissue?

<p>Scurvy (A)</p> Signup and view all the answers

Which disorder involves a platelet count lower than 150,000?

<p>Quantitative disorders (A)</p> Signup and view all the answers

What symptom typically precedes Henoch-Schönlein purpura?

<ul> <li>acute inflammatory reaction with IgA (C)</li> </ul> Signup and view all the answers

Which condition involves vasculitis and increased vascular permeability leading to localized hemorrhage?

<p>Cushing's syndrome (A)</p> Signup and view all the answers

What is the most common non-iatrogenic cause of thrombocytopenia?

<p>Infection-induced thrombocytopenia (C)</p> Signup and view all the answers

What is a key step in evaluating a patient with thrombocytopenia?

<p>Reviewing the peripheral blood smear (B)</p> Signup and view all the answers

What is the role of calcium content in causing pseudothrombocytopenia?

<p>Triggering platelet agglutination (C)</p> Signup and view all the answers

When should a bone marrow examination be recommended in the evaluation of thrombocytopenia?

<p>Urgently needed diagnosis or after unsuccessful less invasive methods (C)</p> Signup and view all the answers

What is the fundamental defect in Glanzmann's thrombasthenia?

<p>Absence of the platelet GpIIbIIIa receptor (D)</p> Signup and view all the answers

Which condition presents with bleeding in skin, mucous membrane, recurrent epistaxis, and GI hemorrhage?

<p>Glanzmann's thrombasthenia (A)</p> Signup and view all the answers

What therapy is successful in refractory or relapsing TTP?

<p>Rituximab (A)</p> Signup and view all the answers

Which condition is almost always due to iron deficiency, inflammation, cancer, or infection?

<p>Reactive thrombocytosis (D)</p> Signup and view all the answers

What is the main reason for initiating plasma exchange in TTP?

<p>To resolve hemolysis signs (A)</p> Signup and view all the answers

Which therapy should only be used as an adjunct to plasma exchange in TTP?

<p>Glucocorticoids (D)</p> Signup and view all the answers

What is a common characteristic of Drug-Induced Thrombocytopenia?

<p>It may occur due to herbal and over-the-counter preparations. (A)</p> Signup and view all the answers

Which drug may cause thrombocytopenia within 24 hours of initial exposure?

<p>Abciximab (B)</p> Signup and view all the answers

How long after the initial exposure to classic drug-dependent antibodies does thrombocytopenia typically occur?

<p>21 days (D)</p> Signup and view all the answers

What is a distinguishing feature of the thrombocytopenia caused by platelet GpIIbIIIa inhibitory drugs like abciximab?

<p>It may occur within 24 hours of initial exposure. (B)</p> Signup and view all the answers

What is a distinguishing characteristic of Heparin-Induced Thrombocytopenia?

<p>It is one of the most prescribed drugs. (B)</p> Signup and view all the answers

Why should all drugs be suspected in a patient with thrombocytopenia without an apparent cause?

<p>Because any drug could potentially be the cause and should be stopped. (D)</p> Signup and view all the answers

Flashcards

Bleeding Without Anatomical Cause

A functional impairment of the normal hemostatic process is inferred when a patient has bleeding but no anatomical cause can be identified.

Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia, a disorder characterized by frequent bleeding episodes, typically affects the nose and gastrointestinal tract.

Hemarthrosis

Hemarthrosis, characterized by bleeding in the joints, is a common consequence of various conditions.

Abnormal Telangiectatic Capillaries

Hereditary hemorrhagic telangiectasia involves abnormal telangiectatic capillaries, these fragile vessels easily rupture, resulting in repeated bleeding episodes.

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Pulmonary AVM in HT

A significant portion of individuals with hereditary hemorrhagic telangiectasia (HT) develop pulmonary arteriovenous malformations (AVM), specifically around 40% of cases.

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Epistaxis in HT

Nosebleeds, or epistaxis, in individuals with hereditary hemorrhagic telangiectasia usually begin after the age of 12, often as a prominent symptom.

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Scurvy: Vitamin C Deficiency

Scurvy, a nutritional deficiency, arises from a lack of vitamin C, which is necessary for the production of hydroxyproline, a key component of collagen.

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Henoch-Schönlein Purpura: Inflammatory Reaction

Henoch-Schönlein purpura (HSP), a condition affecting primarily children, involves an acute inflammatory reaction characterized by IgA and complement components present in capillaries, mesangial tissues, and small arterioles.

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Glucocorticoid Therapy in Scurvy

In cases of scurvy, prolonged glucocorticoid therapy can worsen existing bleeding and bruising tendencies due to weakened connective tissue.

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Quantitative Disorders: Low Platelet Count

Quantitative disorders refer to a platelet count below 150,000, often causing bleeding problems.

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HSP: Preceding Inflammatory Reaction

Henoch-Schönlein purpura is typically preceded by an acute inflammatory reaction involving IgA (immunoglobulin A).

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Cushing's Syndrome: Vasculitis and Bleeding

Cushing's syndrome, a condition resulting from prolonged exposure to high levels of cortisol, involves vasculitis and increased vessel permeability, leading to localized bleeding.

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Infection-Induced Thrombocytopenia

Infection-induced thrombocytopenia is the most common non-iatrogenic cause of thrombocytopenia, meaning it is not caused by medical treatment.

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Peripheral Blood Smear in Thrombocytopenia

A critical step in evaluating thrombocytopenia involves examining the peripheral blood smear, a test that assesses the appearance of blood cells in the peripheral blood.

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Pseudothrombocytopenia: High Calcium Trigger

Pseudothrombocytopenia, a condition where the platelet count appears low due to platelet agglutination, is often triggered by high levels of calcium in the blood.

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Bone Marrow Examination in Thrombocytopenia

A bone marrow examination, a more invasive procedure, is recommended in the evaluation of thrombocytopenia when a diagnosis is urgently needed or less invasive methods have been unsuccessful.

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Glanzmann's Thrombasthenia: GpIIbIIIa Receptor Defect

Glanzmann's thrombasthenia involves a fundamental defect in the platelet GpIIbIIIa receptor, which plays a crucial role in platelet aggregation.

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Glanzmann's Thrombasthenia: Bleeding Symptoms

Glanzmann's thrombasthenia, a rare bleeding disorder, presents with bleeding in the skin, mucous membranes, recurrent nosebleeds, and gastrointestinal hemorrhage.

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Rituximab for Refractory TTP

Rituximab, a monoclonal antibody targeting B cells, proves effective in treating refractory or relapsing thrombotic thrombocytopenic purpura (TTP).

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Reactive Thrombocytosis: Underlying Causes

Reactive thrombocytosis, a condition where platelets increase in number, is often linked to iron deficiency, inflammation, cancer, or infection.

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Plasma Exchange in TTP

Plasma exchange, a procedure that removes plasma from the blood and replaces it with fresh frozen plasma, is crucial in TTP to address hemolysis, a condition where red blood cells are prematurely destroyed.

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Glucocorticoids in TTP

Glucocorticoids, while useful for managing inflammation in TTP, should not be used as the primary treatment but rather an adjunct to plasma exchange.

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Drug-Induced Thrombocytopenia: Any Drug

Drug-induced thrombocytopenia can occur as a result of exposure to various medications, including herbal and over-the-counter preparations.

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Abciximab: Rapid Thrombocytopenia

Abciximab, a drug that inhibits platelet aggregation, can cause thrombocytopenia within 24 hours of initial exposure.

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Classic Drug-Dependent Antibodies: 21-Day Delay

Classic drug-dependent antibodies often cause thrombocytopenia 21 days after initial exposure to the drug.

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GpIIbIIIa Inhibitory Drugs: Rapid Effect

Thrombocytopenia caused by drugs that inhibit the GpIIbIIIa receptor, like abciximab, can occur within 24 hours of initial exposure.

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Heparin-Induced Thrombocytopenia (HIT)

Heparin-induced thrombocytopenia (HIT), a potentially severe condition, involves the immune system reacting to heparin, a commonly prescribed drug.

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Drugs as Potential Culprits

In thrombocytopenia without an apparent cause, all drugs should be considered as potential culprits and should be discontinued.

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