Immune Thrombocytopenia vs Thrombotic Thrombocytopenic Purpura Flashcards

1
Q

What is the epidemiology of ITP?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the pathogenesis of ITP?

A

Destruction of platelets by autoantibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the clinical presentations of ITP?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What does a peripheral blood film show in ITP?

A

Abnormal platelets and all other cells normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What does a bone marrow biopsy show in ITP?

A

High number of megakaryocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does FBC show in ITP?

A

Thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the results of lab coagulation screens in ITP?

A

PT, APTT, TT are within normal range

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the epidemiology of TTP?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the pathogenesis of TTP?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the classic pentad of symptoms in TTP?

A
  • Fever
  • Anaemic symptoms (due to Microangiopathic Haemolytic Anaemia (MAHA))
  • Renal impairment
  • Fluctuating neurological signs
  • Bleeding/bruising easily (due to thrombocytopenia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does a bone marrow biopsy show in TTP?

A

Schistocytes, poikilocytes (occur in MAHA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What does FBC show in TTP?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the results of lab coagulation screens in TTP?

A
  • PT normal/increased
  • APTT normal/increased
  • Fibrinogen and antithrombin levels normal
  • FDP/D-Dimer level elevated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is Evan’s syndrome?

A

ITP occurs with warm AIHA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the treatment for ITP?

A

Corticosteroids with/without IV immunoglobulins.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the diagnostic methods for TTP?

A

Pentad of symptoms and blood film findings.

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

17
Q

What is the treatment for TTP?

A

Plasma exchange with high-dose corticosteroid therapy.

18
Q

Why is TTP considered a haematological emergency?

A

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

19
Q

What is thrombocytopenia?

A

Platelet count below normal range

20
Q

What causes underproduction of platelets?

A

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

21
Q

What conditions can lead to reduced survival of platelets?

A

Conditions affecting peripheral circulation

22
Q

What autoimmune condition causes Immune Thrombocytopenia (ITP)?

A

Autoimmune conditions

23
Q

What is hypersplenism?

A

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

24
Q

What drugs can affect platelet survival?

A

NSAIDs, penicillin, furosemide

25
Q

What infections can lead to reduced platelet survival?

A

Infection, inflammation, and sepsis, especially when causing DIC

26
Q

What is Thrombotic Thrombocytopenic Purpura (TTP)?

A

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

27
Q

What is pseudothrombocytopenia?

A

In vitro phenomenon of a falsely reported low platelet count, typically caused by EDTA-dependent platelet aggregation

Ethylenediaminetetraacetic acid

28
Q

Give 3 examples of anticoagulants that do not cause pseudothrombocytopenia?

A

Lithium heparin

Sodium oxalate

Sodium citrate

29
Q

What is the characteristic presentation of pseudothrombocytopenia?

A

Low platelet count on FBC

No petechiae, no mucosal bleeding