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Hematology > Inherited And Acquired Vessel Wall Disorders > Flashcards

Inherited And Acquired Vessel Wall Disorders Flashcards

1
Q

five major components In hemostatic response

A 
Platelets
Coagulation factors
Coagulation inhibitors 
Fibrinolysis 
Blood vessels
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2
Q

What is primary hemostasis

A

Vasoconstriction and platelet adhesion and aggregation leading to platelet plug formation

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3
Q

Secondary hemostasis

A

Coagulation system activation

Fibrin strand and platelet plug reinforcement

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4
Q

Fibrinolysis

A

Activation of fibrin bound plasminogen

Clot lysis

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5
Q

Vessel wall layers

A

Intima
Média
Adventitia

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6
Q

Component of intima layer

A

Endothelial cells

Basement membrane of sub endothelial micro fibrils with collagen + elastin

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7
Q

Média layer components

A

Circular smooth muscles
Collagen fibers
External elastic lamina

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8
Q

Role of smooth muscle in média layer

A

Contraction or relaxation which regulates rate and pressure of blood flow

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9
Q

Elastin role in blood vessel

A

Stretch and recoil

Tone

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10
Q

Adventitia layer blood vessel

A

Collagen fibers

Fibroblasts

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11
Q

Role of Adventitia layer

A

Protection

Anchor in surrounding structures

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12
Q

Hereditary vascular causes of bleeding

A

Hereditary hemorrhagic telangiectasia
Ehlers danois syndrome
Marfan syndrome

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13
Q

Metabolic, inflammatory vascular causes of bleeding

A 
HEnoch schonlein syndrome 
Scurvy Amyloid
Steroid purpura
Sénile purpura
Rickettsial diseases 
Polyclonal gammopathies
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14
Q

Hereditary hemorrhagic telangiectasia inheritance

A

Autosomal dominant trait

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15
Q

What type of genetic defect in Hereditary hemorrhagic telangiectasia

A

Endothelial protein Endoglin mutation

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16
Q

What are telangiectasia in Hereditary hemorrhagic telangiectasia

A

Dilated micro vascular swelling

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17
Q

Where do telangiectasia develop

A

Skin
Mucous membranes
Internal organs

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18
Q

Types of shunts visible in Hereditary hemorrhagic telangiectasia

A

Pulmonary
Hepatic
Splenic
Cerebral

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19
Q

Recurrent epistaxes and gastrointestinal tract hemorrhage common in Hereditary hemorrhagic telangiectasia

A

Yes

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20
Q

Treatment of Hereditary hemorrhagic telangiectasia

A 
Embolization
 laser treatments 
estrogen 
tranexamic acid
 iron supplement
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21
Q

Treatment used to reduce gastrointestinal bleeding in severe cases in Hereditary hemorrhagic telangiectasia

A

Thalidomide
Lenalinomide
Bevcizumab

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22
Q

What is senile purpura

A

Simple easy bruising disorder occurring mostly in healthy woman especially of childbearing age

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23
Q

Where do you mostly see senile Purpera caused by atrophy of supporting tissues of cutaneous blood vessels

A

Dorsal aspect of the forearms and hands

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24
Q

What are the causes of Purpura associated with infection

A

Damage by the organism to the blood vessels
disseminated intravascular coagulation
immune complex formation

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25
Q

What is henoch schonlein syndrome

A

Purpuric rash with localized edema and itching especially on the buttocks and extensor surfaces of the lower legs and elbows

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26
Q

Who is mostly concerned by henoch schonlein syndrome

A

Children

people after acute upper respiratory tract infection

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27
Q

Which immunoglobulin he’s concerned by vasculitis in henoch schonlein syndrome

A

IgA

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28
Q

What are the symptoms of henoch schonlein syndrome

A 
Purpuric rash 
localized edema 
itching 
painful joint swelling
 hematuria 
abdominal pain
29
Q

Is henoch schonlein syndrome Self-limiting or extensive

A

Self-limiting

30
Q

What is curvy and what causes it

A

Vitamin C deficiency leads to defective collagen which cause Perifollicular petechiae ,bruising and mucosal hemorrhage

31
Q

What happens in steroids purpura

A

There is defective vascular supportive tissue

32
Q

What drugs do you use in Bleeding fromvascular disorder or thrombocytopenia

A

Tranexamic acid

Aminocaproic acid

33
Q

What are platelets

A

Small anucleated cells that play critical role in hemostasis and thrombosis

34
Q

When are platelets in high quantity in bloodstream and why

A

When there is damage to the vessel wall so that there is vascular plug and occlusion of the side of damage

35
Q

Number of platelets produced from one megakaryocytes

A

1000 to 2000 platelets let

36
Q

How long do platelet last in blood circulation

A

10 days

37
Q

What are the roles of platelets in hemostasis

A

Release hemostatic proteins
platelet adhesion
platelet aggregation

38
Q

Two types of platelet disorder class

A

quantitative disorders

qualitative disorders

39
Q

What are the platelets quantitative disorders

A

ANormal distribution
Dilution effect
decreased production
increased destruction

40
Q

What are the qualitative disorders of platelets

A

Inherited disorders

Acquired Disorders due to medication ,chronic renal failure ,cardiopulmonary bypass

41
Q

What is thrombocytopenia

A

Low platelet counts

42
Q

What are some examples of immune mediated thrombocytopenia

A 
Idiopathic 
drug induced 
collagen vascular disease
 lymphoproliferative disease
 sarcoidosis
43
Q

What are some examples of non-immune mediated thrombocytopenia

A

Disseminated intravascular coagulation

microanngiopathic hemolytic anemia

44
Q

What are some hereditary functional platelet disorder’s

A

Glanzmanns thrombasthenia
Bernard soulier syndrome
Storage pool disease

45
Q

Glanzmanns thrombasthenia. Type of inheritance

A

A recessive

46
Q

what are the characteristics of Glanzmanns thrombasthenia

A

Prolonged bleeding time
failed aggregation of platelets with ADP adrenaline collagen or thrombin
Deficiency of platelet membrane GP IIb - IIIa ( fibrin can’t bind )

47
Q

Type of inheritance in Bernard soulier syndrome

A

A recessive

48
Q

Characteristics of Bernard soulier syndrome

A

Deficiency of platelets membrane GP Ib - IX so Defective binding of blood clotting proteins von Willebrand factor

49
Q

What do you see in the peripheral blood smear of Bernard soulier syndrome

A

Giant platelets

50
Q

Is there a response to ristocetin in Bernard soulier syndrome

A

No

51
Q

Type of inheritance in storage pool disease

A

Dominant

52
Q

storage pool disease Characteristics

A

Platelet storage granules are decreased in number and content
Abnormal granules formation in megakaryocytes
Mild bleeding
Decreased platelet aggregation because low secretion of ADP
Sometimes oculocutaneous albinism

53
Q

Characteristics of bleeding disorders of platelets or vessels

A 
Skin ,mucous membrane site of bleeding
Petechiae
Small ecchymoses 
Rare hemarthrosis or muscle bleeding
Bleeding after cuts and scratches
Mild Immediate bleeding after surgery or trauma
54
Q

Coagulation factor disorders characteristics

A 
Site of bleeding is deep in soft tissues
 No Petechiae 
Large and deep ecchymoses 
Large hemarthrosis and muscle bleeding
No bleeding after cuts and scratches 
severe and delayed bleeding after surgery or trauma
55
Q

See diagnostic approach chart

A
56
Q

Most common immune destructive thrombocytopenia in children and adults

A

ITP

57
Q

What is itp

A

Auto immune disorder with antibodies produced against platelet surface glycoproteins resulting in clearance of platelets from circulation

58
Q

In children is itp commonly chronic or acute

A

Acute and resolves spontane

59
Q

In adult is ITP commonly chronic or acute

A

Chronic with insidious onset

60
Q

2 diagnostic criteria for ITP

A

Thrombocytopenia with normal blood counts

No clinical condition associated associated with thrombocytopenia

61
Q

Two pathways for itp

A

Only peripheral platelet destruction

Platelet distraction with inhibition of megakaryocytes platelet production

62
Q

Clinical manifestation of itp

A

Marked variability between patient
Severe bleeding to only petechiae
Mucocutaneous bleeding
Intracranial hemorrhage uncommon

63
Q

Do you treat itp in which there is more than 50,000 platelet counts and no symptoms

A

No

64
Q

Do you treat itp in which there is 20 to 50,000 platelets with no bleeding

A

No

65
Q

Do you treats itp with 20 to 50,000 platelets in which there is bleeding

A

Yes with steroids or IVIG

66
Q

Do you treat ITP with less than 20,000 platelets with no bleeding

A

Yes with steroids

67
Q

Do you treat ATP in which there is less than 20,000 platelets with bleeding

A

Yes with IV I G

68
Q

Steroids used in ITP

A

Prednisolone
dexamethasone
Methyleprednisone pulse therapy

69
Q

Other form of treatment of itp

A 
Steroids 
splenectomy
 intravenous immunoglobulin IVIG 
immunosuppressive drugs like mycophenolate , azathioprine
 rituximab 
Thrombopoieisis stimulating agents
 recombinant FVIIa

Hematology (24 decks)

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