Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is Haemophilia A?

A

Factor VIII deficiency, Sex linked recessive, missense or frameshift mutations or deletions

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

What is Haemophilia B?

A

Factor IX deficiency

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

Where is Factor VII synthesized?

A

Liver and endothelial cells

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

Which mutation leads to the more severe form of Haemophillia A?

A

Flip tip inversion in the Factor VIII gene?

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

What are the clinical features of Haemophilia A?

A
  1. Recurrent painful Haemarthroses
  2. Muscle haematomas
  3. Can progress to joint deformity
  4. Prolonged bleeding after dental procedures
  5. Spontaneous G.I bleeds and haematuria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are Haemophilic pseudotumours?

A

Large encapsulated haematomas withh progressive cystic swelling from repeated hemorrhage. Best visualized by MRI

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

What are the treatments available for haemophilia A patients?

A
  1. Bleeding episodes are treated with Factor VIII replacement therapy.
  2. Recombinant factor VII and plasma derived factor VII preparations are available for clinical use
  3. 1-Diamino-8-D-Arginine vasopressin (DDAVP) provides an alternative means of increasing the plasma factor VIII level in milder haemophiliacs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a side effect of DDAVP?

A

Antidiuretic action

2.Avoid in the elserly

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

What recommendation is made to Haemophiliacs?

A

Have regular conservative dental care.

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

What is one of the most serious complications with haemophilia treatment?

A

The development of antibodies to to infused Factor VIII (occurs in 30-40% of patients)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How can the antibody development to infused Factor VIII be countered?

A

Immunosupression

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

What is the difference in the synthesis of factor VIII and IX?

A

Factor IX synthesis is dependent on vitamin K

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

What test are abnormal in a patient with Haemophilis B.

A
  1. PTT

2. Factor IX clotting assay

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is von Willebrand disease?

A

A disorder in which there are low levels or abnormal function of VWF stemming from a missense mutation or null mutation

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

Where is VWF produced?

A
  1. Endothelial cells

2. Platelets

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

What are the 2 functions of VWF?

A
  1. Promotes platelet adhesion to subendothelium at high shear rates
  2. It is the carrier molecule for Factor VIII protecting it from premature destruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What other factor is reduced in von Willebrand disease?

A

Factor VIII

18
Q

What is the most inherited bleeding disorder?

A

Von Willebrand factor disease

19
Q

What is the usual inheritance patern for Von Willebrand disease?

A

Autosomal Dominant

20
Q

Which gender is generally worse affected at a given VWF level?

A

Women

21
Q

Lab findings?

A

1.Factor VIII - Low
2.PTT elevated
3.VWF levels low
.Platelet count normal except intype 2B disease

22
Q

Is senile purpura a bleeding disorder?

A

No, comes with aging

23
Q

What is andiodysplasia?

A

Structural insufficiency of blood vessel (small) generally leads to G.I bleeding

24
Q

What is Hereditary hemmorhagic telangiectasia?

A

Structural deficiency of blood vessels these can be spotted around the lips and nose.

25
Q

When can a diagnosis on Henoch-Schonlein purpura be made?

A

When a perpuric rash is associated with fever, malaise, polyarthralgia and colicky abdominal pain.
2.Thought to be an immune mediated vasculitis

26
Q

What kind bleeding would one expect with “production failure” thrombocytopenia?

A

Superficial/Surface i.e petechiae etc

27
Q

What usually precedes ITP in children.

A

Viral infection

28
Q

What is the treatment for ITP in children?

A

Self Limiting, goes away within weeks

29
Q

Is ITP in adults preceded by viral infection?

A

NO

30
Q

What is the treatment for ITP in adults?

A

Immunosuppresion

31
Q

What is HIT?

A

Heparin induce thrombocytopenia

32
Q

What is the pathogenesis of HIT?

A
  1. Antibodies develop to Heparin-PF4 complex
  2. Antibody+complex binds to the platelets FC receptor
  3. causes platelet activation and aggregation
33
Q

What is the classic presentation for HIT?

A

1.Thrombosis
2.Thrombocytopenia from increased use of platelets
Platelets are the only abnormal cell

34
Q

How can diagnosis be made?

A

Ru ELISA to test for Heparin-P4 antibody

35
Q

What is (TTP) Thrombotic Thrombocytopenic purpura?

A

Inherited or acquired deficiency of ADAMTS13

2.Aquired is an IgG antibody against ADAMTS13

36
Q

What is the pathogenesis?

A

ULVWF not able to be cleaved which binds platelets via Ib+IIb/IIIa which leads to occlusion due to platelet aggregation which leads to microangiopathic anemia

37
Q

What is the presentation of TTP?

A
  1. Renal insufficiency
  2. thrombocytopenia
  3. Microangipathic hemolytic anemia
  4. neurological changes
  5. fever
38
Q

What is another blood smear finding indicative of TTP?

A

Schistocytes Increased LDH

39
Q

What is the treatment for TTP?

A

Plasma exchange NOT PLATELETS.

40
Q

Injuries around the face of a Haemophiliac are particularly worrisome because?

A

Continued bleeding into tissue could obstruct the larynx and kill the patient.

Haematology (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions