Inherited and acquired vessel wall disorders

Question 1

Layers of vessel wall

Answer 1

Intima- innermost, covered by endothelium
Media-middle layer containing circularly arranged smooth muscle cells, collagen fibres
Adventitia - outermost , collagen and fibroblasts

Question 2

Hereditary causes of Vascular bleeding

Answer 2

1. Hereditary hemorrhagic telangectasia
2. Ehler- Danlos syndrome
3. Marfans syndrome

Question 3

Metabolic and inflammatory causes of vascular bleeding

Answer 3

1. Henoch schonlein syndrome
2. Scurvy amyloid
3. Steroid purpura
4. Senile purpura
5. Rickettsial diseases
6. Polyclonal gammopathies

Question 4

Method of inheritance of hereditary hemorrhagic telangectasia

Answer 4

Autosomal dominant

Question 5

Pathophysiology of HHT

Answer 5

1. Genetic defect that lead to mutations in endothelial protein endoglin
2. This leads to dilated microvascular swellings known as telangiectasia which appear in childhood and become numerous in adulthood
3. The microvascular swellings appear in the skin, internal organs and mucous membranes

Question 6

Other signs present in HHT

Answer 6

1. Pulmonary, hepatic, splenic, cerebral arteriovenous shunts
2. Recurrent epistaxis
3. Recurrent GI hemorrhage may cause chronic IDA

Question 7

Treatment of HHT

Answer 7

Embolization- block where dilatations are
Laser treatment
estrogen
tranexamic acid
iron supplementation
Thalidomide
Lenalidomide
Bevacizumab

Question 8

What causes senile purpura

Answer 8

Atrophy of the supporting tissues of cutaneous blood vessels mainly in dorsal aspect of forearm and hand

Question 9

What age group does senile purpura occur in

Answer 9

Older people

Question 10

Microorganisms and infections can cause purpura by

Answer 10

Purpura from vascular damage by organism
DIC
Immune complex formation

Question 11

Henoch schonlein syndrome is usually seen in

Answer 11

Children

Question 12

Henoch schonlein syndrome usually follows

Answer 12

An acute upper respiratory tract infection

Question 13

Henoch schonlein syndrome is mediated by this antibody

Answer 13

igA mediated vasculitis

Question 14

Characteristics of Henoch Schonlein syndrome

Answer 14

1. Purpuric rash
2. Localised edema
3. Itching

Question 15

On which parts of the body are these characteristic properties of henoch scholeins dx found

Answer 15

Buttocks
Extensor surfaces of lower legs
Elbows

Question 16

Other physical characteristics that may occur

Answer 16

Painful joint swelling
Haematuria
Abdominal pain

Occasionally kidney failuire

Question 17

Henoch schonlein syndrome prognosis

Answer 17

Self limiting

Question 18

Scurvy pathophysiology

Answer 18

Vitamin C is involved in formation of collagen. A deficiency in it will affect integrity of the vessel wall causing bruising , petechiae

Question 19

Steroid purpura pathophysiology

Answer 19

Associated with long term steroid therapy usually in people with autoimmune diseases or cusions syndrome leading to a defect in vascular supporting tissues

Question 20

Antifibrinolytic drugs that can be used in vascular disorders and thrombocytopenia

Answer 20

Tranexamic acid

Aminocaproic acid

Question 21

Classification of platelet disorderd

Answer 21

1. Quantitative - Has to do with number of platelets

2. Qualitative - Inherited and acquired disorders

Question 22

Hereditary functional platelet disorders

Answer 22

Glanzmanns thrombastenia

Bernard soulier syndrome

Storage pool disease

Question 23

Type of inheritance in glanzmanns thrombastenia

Answer 23

Autosomal recessive

Question 24

Characteristics of glanzmanns thrombastenia

Answer 24

Prolonged bleeding time test
Platelets fail to aggregate with ADP , collagen, adrenaline, thrombin
Deficiency of platelet membrane GP2b-3a

Question 25

Peripheral blood smear in Bernard soulier syndrome shows

Answer 25

Giant platelets, as big as RBC

Question 26

Pathophysiology of glanzmanns thrombastenia

Answer 26

Problem with platelet aggregation due to defect in GP2b-3a

Question 27

Method of inheritance in bernard soulier syndrome

Answer 27

Autosomal recessive mode of inheritance

Question 28

Pathophysiology of Bernard soulier syndrome

Answer 28

Deficiency of platelet membrane GP1b causing deficient binding with vWf

Question 29

Method of inheritance of atorage pool disease

Answer 29

Autosomal dominant inheritance

Question 30

Pathophysiology of storage pool disease

Answer 30

Platelet granules are reduced in number and content | Abnormal formation of granules in megakaryocytes

Question 31

Characteristics of storage pool diseases

Answer 31

1. Mild bleeding in women 2. Decreased platelet aggregation due to low secretion of ADP 2. associated sometimes with oculocutaneous albinism

Question 32

Bleeding in skin and mucous membrane is characteristic of this bleeding disorder

Answer 32

Platelet disorder

Question 33

Bleeding into deep soft tissues like joints and muscles denotes

Answer 33

Coagulation factor disorders or secondary hemostasis disorders

Question 34

Prescence of petechiae shows this bleeding disorder

Answer 34

platelet disorder

Question 35

No petechiae shows that this bleeding disorder is absent

Answer 35

Coagulation factor disorder

Question 36

Ecchymoses in platelet disorders are

Answer 36

small | superficial

Question 37

Ecchymoses in coagu;ation factor disorders are

Answer 37

Large and deep

Question 38

Muscle bleeding in platelet disorders are

Answer 38

Very rare

Question 39

Hemarthrosis in coagulation factor disorders are

Answer 39

common

Question 40

Bleeding after cuts and scratches is seen in

Answer 40

Platelet disorders only

Question 41

Bleeding after surgery or trauma in platelet disorders is

Answer 41

Extremely mild, immediate

Question 42

Bleeding after surgery or trauma when you have coagulation factor disorders coagulation

Answer 42

Are delayed for 1-2 days and often severe

Question 43

Diagnostic approach to patient with mucocutaneous bleeding

Answer 43

1. full blood count | 2. Examination of peripheral blood smear

Question 44

If examination of peripheral smear yielded low platelet count with large platelets the following conditions come to mind

Answer 44

ITP | Bernard-soulier

Question 45

If peripheral smear exam shows low platelet count with normal sized platelets

Answer 45

Aplastic anemia Amegakaryocytic thrombocytopenia Leukemia

Question 46

If peripheral smear shows Normal platelet counts with isolated platelets

Answer 46

Glanzmanns Thrombastenia

Question 47

If peripheral smear shows normal platelets in clumps

Answer 47

von willebrands disease platelet function disorders

Question 48

Immune thrombocytopenic purpura

Answer 48

A situation where body produces autoantibodies against certain antigens on the platelets leading to destruction and low platelet counts

Question 49

Most common immune destructive thrombocytopenia in adults and children

Answer 49

ITP

Question 50

In children ITP is

Answer 50

Acute resolves on its own follows a viral infection Affects both gender in equal proportions

Question 51

In adults ITP is

Answer 51

Chronic Usually diagnosed incidentally More expressed in females

Question 52

Diagnostic criteria for ITP

Answer 52

Thrombocytopenia with normal blood counts No clinically apparent diseases that can cause thrombocytopenia

Question 53

Pathogenesis of PLT

Answer 53

Increased platelet destruction along with inhibition of megakaryocyte platelet production

Question 54

Clinical manifestations of ITP

Answer 54

``` Patient variability Severe Bleeding - easy bruising Mucocutaneous bleeding Uncommon intracranial hemorrhage Compared to vasculitic purpura is asymptomatic and not palpable ```

Question 55

Initial treatment of ITP

Answer 55

>50,000 - no treatment 20-50,000 - if no bleeding nothing; if there is bleeding give steroids and IVIG <20,000 - If not bleeding give steroids If bleeding give IVIG

Question 56

Treatment of ITP

Answer 56

``` Steroids Splenectomy Intravenous immunoglobulin (IVIG) Rituximab Thrombopoiesis stimulating agents Recombinant FV11a ```

Question 57

2nd line treatments for ITP

Answer 57

Recombinant FV11a Splenectomy Thrombopoiesis stimulating agents

Question 58

Evamples of platelet stimulating agents

Answer 58

Romiplostin | Eltrombopag

Question 59

Why are platelet transfusions not a mainstay form of treatment

Answer 59

It leads to further activation of antibodies which cause further destruction of platelets