Immune Thrombocytopenia vs Thrombotic Thrombocytopenic Purpura

Question 1

What is the epidemiology of ITP?

Answer 1

Mostly self-limiting in children but is chronic relapsing in adults

Question 2

What is the pathogenesis of ITP?

Answer 2

Destruction of platelets by autoantibodies

Question 3

What are the clinical presentations of ITP?

Answer 3

Same Pale chize: Small red/purple spots on mucosal membranes, skin, retina, conjunctiva, mucocutaneous bleeding from gums, nose (epistaxis), females have heavy periods

Question 4

What does a peripheral blood film show in ITP?

Answer 4

Abnormal platelets and all other cells normal

Question 5

What does a bone marrow biopsy show in ITP?

Answer 5

High number of megakaryocytes

Question 6

What does FBC show in ITP?

Answer 6

Thrombocytopenia

Question 7

What are the results of lab coagulation screens in ITP?

Answer 7

PT, APTT, TT are within normal range

Question 8

What is the epidemiology of TTP?

Answer 8

Usually affects 20-50 yr olds and is more common in women, life-threatening disease in children and adults

Question 9

What is the pathogenesis of TTP?

Answer 9

Congenital/acquired absence/decrease of the von Willebrand factor-cleaving protease ADAMTS13: Microthrombi formation

Question 10

What is the classic pentad of symptoms in TTP?

Answer 10

• Fever
• Anaemic symptoms (due to Microangiopathic Haemolytic Anaemia (MAHA))
• Renal impairment
• Fluctuating neurological signs
• Bleeding/bruising easily (due to thrombocytopenia)

Question 11

What does a bone marrow biopsy show in TTP?

Answer 11

Schistocytes, poikilocytes (occur in MAHA)

Question 12

What does FBC show in TTP?

Answer 12

Thrombocytopenia, elevated unconjugated bilirubin, elevated LDH, elevated reticulocyte count, decreased haptoglobin concentration

Question 13

What are the results of lab coagulation screens in TTP?

Answer 13

• PT normal/increased
• APTT normal/increased
• Fibrinogen and antithrombin levels normal
• FDP/D-Dimer level elevated

Question 14

What is Evan’s syndrome?

Answer 14

ITP occurs with warm AIHA.

Question 15

What is the treatment for ITP?

Answer 15

Corticosteroids with/without IV immunoglobulins.

Question 16

What are the diagnostic methods for TTP?

Answer 16

Pentad of symptoms and blood film findings.

Can also do ADAMTS13 activity, functional inhibitor, and anti-ADAMTS13 antibody assays.

Question 17

What is the treatment for TTP?

Answer 17

Plasma exchange with high-dose corticosteroid therapy.

Question 18

Why is TTP considered a haematological emergency?

Answer 18

Due to infarction in the microcirculation in the brain, heart, and kidney, which can be fatal.

Question 19

What is thrombocytopenia?

Answer 19

Platelet count below normal range

Question 20

What causes underproduction of platelets?

Answer 20

Drugs affecting haematopoietic stem cell, liver damage reducing TPO synthesis, part of pancytopenia due to failed stem cell or unhealthy microenvironment

Question 21

What conditions can lead to reduced survival of platelets?

Answer 21

Conditions affecting peripheral circulation

Question 22

What autoimmune condition causes Immune Thrombocytopenia (ITP)?

Answer 22

Autoimmune conditions

Question 23

What is hypersplenism?

Answer 23

An overactive spleen that causes most of the platelet mass to be kept in the spleen

Question 24

What drugs can affect platelet survival?

Answer 24

NSAIDs, penicillin, furosemide

Question 25

What infections can lead to reduced platelet survival?

Answer 25

Infection, inflammation, and sepsis, especially when causing DIC

Question 26

What is Thrombotic Thrombocytopenic Purpura (TTP)?

Answer 26

A life-threatening disorder characterized by large vWD multimers causing thrombosis in microcirculation, leading to ischemia in critical organs