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Haematology > Inherited and acquired vessel wall disorders > Flashcards

Inherited and acquired vessel wall disorders Flashcards

1
Q

Layers of vessel wall

A

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

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2
Q

Hereditary causes of Vascular bleeding

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

Metabolic and inflammatory causes of vascular bleeding

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

Method of inheritance of hereditary hemorrhagic telangectasia

A

Autosomal dominant

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5
Q

Pathophysiology of HHT

A
  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
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6
Q

Other signs present in HHT

A
  1. Pulmonary, hepatic, splenic, cerebral arteriovenous shunts
  2. Recurrent epistaxis
  3. Recurrent GI hemorrhage may cause chronic IDA
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7
Q

Treatment of HHT

A 
Embolization- block where dilatations are
Laser treatment
estrogen
tranexamic acid
iron supplementation
Thalidomide
Lenalidomide
Bevacizumab
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8
Q

What causes senile purpura

A

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

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9
Q

What age group does senile purpura occur in

A

Older people

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10
Q

Microorganisms and infections can cause purpura by

A

Purpura from vascular damage by organism
DIC
Immune complex formation

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11
Q

Henoch schonlein syndrome is usually seen in

A

Children

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12
Q

Henoch schonlein syndrome usually follows

A

An acute upper respiratory tract infection

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13
Q

Henoch schonlein syndrome is mediated by this antibody

A

igA mediated vasculitis

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14
Q

Characteristics of Henoch Schonlein syndrome

A
  1. Purpuric rash
  2. Localised edema
  3. Itching
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15
Q

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

A

Buttocks
Extensor surfaces of lower legs
Elbows

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16
Q

Other physical characteristics that may occur

A

Painful joint swelling
Haematuria
Abdominal pain

Occasionally kidney failuire

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17
Q

Henoch schonlein syndrome prognosis

A

Self limiting

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18
Q

Scurvy pathophysiology

A

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

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19
Q

Steroid purpura pathophysiology

A

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

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20
Q

Antifibrinolytic drugs that can be used in vascular disorders and thrombocytopenia

A

Tranexamic acid

Aminocaproic acid

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21
Q

Classification of platelet disorderd

A
  1. Quantitative - Has to do with number of platelets

2. Qualitative - Inherited and acquired disorders

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22
Q

Hereditary functional platelet disorders

A

Glanzmanns thrombastenia

Bernard soulier syndrome

Storage pool disease

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23
Q

Type of inheritance in glanzmanns thrombastenia

A

Autosomal recessive

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24
Q

Characteristics of glanzmanns thrombastenia

A

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

25
Q

Peripheral blood smear in Bernard soulier syndrome shows

A

Giant platelets, as big as RBC

26
Q

Pathophysiology of glanzmanns thrombastenia

A

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

27
Q

Method of inheritance in bernard soulier syndrome

A

Autosomal recessive mode of inheritance

28
Q

Pathophysiology of Bernard soulier syndrome

A

Deficiency of platelet membrane GP1b causing deficient binding with vWf

29
Q

Method of inheritance of atorage pool disease

A

Autosomal dominant inheritance

30
Q

Pathophysiology of storage pool disease

A

Platelet granules are reduced in number and content

Abnormal formation of granules in megakaryocytes

31
Q

Characteristics of storage pool diseases

A
  1. Mild bleeding in women
  2. Decreased platelet aggregation due to low secretion of ADP
  3. associated sometimes with oculocutaneous albinism
32
Q

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

A

Platelet disorder

33
Q

Bleeding into deep soft tissues like joints and muscles denotes

A

Coagulation factor disorders or secondary hemostasis disorders

34
Q

Prescence of petechiae shows this bleeding disorder

A

platelet disorder

35
Q

No petechiae shows that this bleeding disorder is absent

A

Coagulation factor disorder

36
Q

Ecchymoses in platelet disorders are

A

small

superficial

37
Q

Ecchymoses in coagu;ation factor disorders are

A

Large and deep

38
Q

Muscle bleeding in platelet disorders are

A

Very rare

39
Q

Hemarthrosis in coagulation factor disorders are

A

common

40
Q

Bleeding after cuts and scratches is seen in

A

Platelet disorders only

41
Q

Bleeding after surgery or trauma in platelet disorders is

A

Extremely mild, immediate

42
Q

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

A

Are delayed for 1-2 days and often severe

43
Q

Diagnostic approach to patient with mucocutaneous bleeding

A
  1. full blood count

2. Examination of peripheral blood smear

44
Q

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

A

ITP

Bernard-soulier

45
Q

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

A

Aplastic anemia
Amegakaryocytic thrombocytopenia
Leukemia

46
Q

If peripheral smear shows Normal platelet counts with isolated platelets

A

Glanzmanns Thrombastenia

47
Q

If peripheral smear shows normal platelets in clumps

A

von willebrands disease

platelet function disorders

48
Q

Immune thrombocytopenic purpura

A

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

49
Q

Most common immune destructive thrombocytopenia in adults and children

A

ITP

50
Q

In children ITP is

A

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

51
Q

In adults ITP is

A

Chronic
Usually diagnosed incidentally

More expressed in females

52
Q

Diagnostic criteria for ITP

A

Thrombocytopenia with normal blood counts

No clinically apparent diseases that can cause thrombocytopenia

53
Q

Pathogenesis of PLT

A

Increased platelet destruction along with inhibition of megakaryocyte platelet production

54
Q

Clinical manifestations of ITP

A 
Patient variability
Severe Bleeding - easy bruising
Mucocutaneous bleeding
Uncommon intracranial hemorrhage
Compared to vasculitic purpura is asymptomatic and not palpable
55
Q

Initial treatment of ITP

A

> 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

56
Q

Treatment of ITP

A 
Steroids
Splenectomy
Intravenous immunoglobulin (IVIG)
Rituximab
Thrombopoiesis stimulating agents
Recombinant FV11a
57
Q

2nd line treatments for ITP

A

Recombinant FV11a
Splenectomy
Thrombopoiesis stimulating agents

58
Q

Evamples of platelet stimulating agents

A

Romiplostin

Eltrombopag

59
Q

Why are platelet transfusions not a mainstay form of treatment

A

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

Haematology (29 decks)

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