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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
What is henoch schonlein syndrome
Purpuric rash with localized edema and itching especially on the buttocks and extensor surfaces of the lower legs and elbows
26
Who is mostly concerned by henoch schonlein syndrome
Children | people after acute upper respiratory tract infection
27
Which immunoglobulin he’s concerned by vasculitis in henoch schonlein syndrome
IgA
28
What are the symptoms of henoch schonlein syndrome
``` Purpuric rash localized edema itching painful joint swelling hematuria abdominal pain ```
29
Is henoch schonlein syndrome Self-limiting or extensive
Self-limiting
30
What is curvy and what causes it
Vitamin C deficiency leads to defective collagen which cause Perifollicular petechiae ,bruising and mucosal hemorrhage
31
What happens in steroids purpura
There is defective vascular supportive tissue
32
What drugs do you use in Bleeding fromvascular disorder or thrombocytopenia
Tranexamic acid | Aminocaproic acid
33
What are platelets
Small anucleated cells that play critical role in hemostasis and thrombosis
34
When are platelets in high quantity in bloodstream and why
When there is damage to the vessel wall so that there is vascular plug and occlusion of the side of damage
35
Number of platelets produced from one megakaryocytes
1000 to 2000 platelets let
36
How long do platelet last in blood circulation
10 days
37
What are the roles of platelets in hemostasis
Release hemostatic proteins platelet adhesion platelet aggregation
38
Two types of platelet disorder class
quantitative disorders | qualitative disorders
39
What are the platelets quantitative disorders
ANormal distribution Dilution effect decreased production increased destruction
40
What are the qualitative disorders of platelets
Inherited disorders | Acquired Disorders due to medication ,chronic renal failure ,cardiopulmonary bypass
41
What is thrombocytopenia
Low platelet counts
42
What are some examples of immune mediated thrombocytopenia
``` Idiopathic drug induced collagen vascular disease lymphoproliferative disease sarcoidosis ```
43
What are some examples of non-immune mediated thrombocytopenia
Disseminated intravascular coagulation | microanngiopathic hemolytic anemia
44
What are some hereditary functional platelet disorder’s
Glanzmanns thrombasthenia Bernard soulier syndrome Storage pool disease
45
Glanzmanns thrombasthenia. Type of inheritance
A recessive
46
what are the characteristics of Glanzmanns thrombasthenia
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
Type of inheritance in Bernard soulier syndrome
A recessive
48
Characteristics of Bernard soulier syndrome
Deficiency of platelets membrane GP Ib - IX so Defective binding of blood clotting proteins von Willebrand factor
49
What do you see in the peripheral blood smear of Bernard soulier syndrome
Giant platelets
50
Is there a response to ristocetin in Bernard soulier syndrome
No
51
Type of inheritance in storage pool disease
Dominant
52
storage pool disease Characteristics
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
Characteristics of bleeding disorders of platelets or vessels
``` 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
Coagulation factor disorders characteristics
``` 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
See diagnostic approach chart
56
Most common immune destructive thrombocytopenia in children and adults
ITP
57
What is itp
Auto immune disorder with antibodies produced against platelet surface glycoproteins resulting in clearance of platelets from circulation
58
In children is itp commonly chronic or acute
Acute and resolves spontane
59
In adult is ITP commonly chronic or acute
Chronic with insidious onset
60
2 diagnostic criteria for ITP
Thrombocytopenia with normal blood counts | No clinical condition associated associated with thrombocytopenia
61
Two pathways for itp
Only peripheral platelet destruction Platelet distraction with inhibition of megakaryocytes platelet production
62
Clinical manifestation of itp
Marked variability between patient Severe bleeding to only petechiae Mucocutaneous bleeding Intracranial hemorrhage uncommon
63
Do you treat itp in which there is more than 50,000 platelet counts and no symptoms
No
64
Do you treat itp in which there is 20 to 50,000 platelets with no bleeding
No
65
Do you treats itp with 20 to 50,000 platelets in which there is bleeding
Yes with steroids or IVIG
66
Do you treat ITP with less than 20,000 platelets with no bleeding
Yes with steroids
67
Do you treat ATP in which there is less than 20,000 platelets with bleeding
Yes with IV I G
68
Steroids used in ITP
Prednisolone dexamethasone Methyleprednisone pulse therapy
69
Other form of treatment of itp
``` Steroids splenectomy intravenous immunoglobulin IVIG immunosuppressive drugs like mycophenolate , azathioprine rituximab Thrombopoieisis stimulating agents recombinant FVIIa ```

Hematology (24 decks)

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