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Systemic Pathology Test 2 > Coagulation 2 > Flashcards

Coagulation 2 Flashcards

1
Q

Platelets are produced from?

A

Cytoplasmic projections of megakaryocytes

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2
Q

Production of platelets is regulated by? What receptor does this interact with?

A

Thrombopoietin which interacts with the c-MPL receptor

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3
Q

Where is thrombopoietin produced?

A

The liver and bone marrow stromal cells

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4
Q

How long do platelets circulate for?

A

7-10 days

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5
Q

What 3 things are secreted by platelets once activated?

A
  1. Dense granules
  2. Alpha granules
  3. Acid hydrolase
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6
Q

What is the role of TXA2?

A

Prevents further activation by the agonist

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7
Q

What inherited disorders can cause platelet issues?

A

Albinism, deafness, and nephritis

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8
Q

What systemic diseases can lead to platelet issues?

A

Renal, hepatic, and myeloproliferative disease

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9
Q

What are the two types of quantitative disorders?

A

Thrombocytopenia where the count is reduced

Thrombocytosis where the count is raised

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10
Q

What 3 things can cause thrombocytopenia?

A

Decreased production, increased destruction, and splenomegaly

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11
Q

What can cause thrombocytosis?

A

Reactive to infection and autonomous

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12
Q

What is the normal range for platelet count?

A

150000-400000

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13
Q

What is pseudothrombocytopenia?

A

When there is clumping of platelets in the presence of EDTA leading to a lower count. Caused by EDTA-dependent antibodies

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14
Q

What 3 things can cause thrombocytopenia?

A
  1. Marrow replacement
  2. Marrow failure as seen in aplastic anemia where fibrosis takes over
  3. Marrow injury from radiation or drugs
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15
Q

What are the 2 types of inherited thrombocytopenias with small platelets?

A

Wiskott-Aldrich and X-linked thrombocytopneia

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16
Q

What is the main thrombocytopenia with normal sized platelets?

A

Familial platelet disorder with predisposition to AML

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17
Q

What are 2 types of thrombocytopenia with large platelets?

A

Bernard-Soulier and grey platelet syndrome

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18
Q

What is the most common thrombocytopenia due to increased destruction>

A

Immune thrombocytopenic purpura

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19
Q

What 4 drugs can cause increased platelet destruction?

A

Heparin, quinidine, vancomycin, and GPIIb antagonists

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20
Q

Post transfusion purpura occurs in those lacking what antigen?

A

HPA-1a antigen

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21
Q

Most cases of immune thrombocytopenic purpura are?

A

Acute

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22
Q

Acute ITP is usually preceded by?

A

Viral infection

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23
Q

Does chronic ITP have something preceding it?

A

No insidious onset

24
Q

In isolated thrombocytopenia platelets count will be? What other findings?

A

Less than 100,000 and they will be large.

Increased megakaryocytes in bone marrow and increased platelet associated IgG

25
Q

Heparin caused what type of thrombocytopenia? This is most common with what type of heparin?

A

Immune mediated

Heparin of high molecular weight

26
Q

The reduced count results in?

A

Venous and arterial thrombosis, limb loss, death

27
Q

How do you treat heparin induced thrombocyto?

A

Discontinue heparin and use a thrombin inhibitor like argatroban or recombinant huridin (refludan)

28
Q

What are the 3 non-imune associated thrombocytopenias?

A
  1. DIC
  2. Hemolytic uremic syndrome
  3. Thrombotic thrombocytopenic purpura
29
Q

What causes TTP?

A

Reduced ADAMTS-13 metalloprotease activity

30
Q

How does heparin cause problems?

A

Binds to PF4 on the platelet leading to an Ig form in against the complex

31
Q

Hemolytic uremic syndrome occurs in?

A

Infants and young children

32
Q

What does hemolytic uremic syndrome cause? What causes it?

A

Bloody diarrhea

Infection by E. Coli O157:H7

33
Q

How does hemolytic uremic syndrome differ from TTP?

A

Less pronounced neurologic symptoms and more pronounced renal symptoms. There is normal ADAMTS13

34
Q

How is hemolytic uremic syndrome treated?

A

Dialysis

35
Q

What are the two types of congenital qualitative disorders?

A

Disorders of membrane glycoprotein and disorders of intracellular proteins

36
Q

How is Bernard Soulier syndrome inherited and what does it cause?

A

Autosomal recessive, leads to abnormal platelet adhesion

37
Q

There is a defect in what gene in Bernard Soulier?

A

GP1b gene

38
Q

How is Bernard Soulier treated?

A

DDAVP or PLT transfusion

39
Q

Other than Bernard Soulier what other disorder can cause aggregation problems?

A

Glanzmann thrombasthenia

40
Q

How is TTP treated?

A

Plasmapheresis and steroids

41
Q

How is hemolytic uremia treated?

A

Dialysis

42
Q

Describe the appearance of platelets in Bernard Soulier.

A

Giant platelets with a pseudonucleus

43
Q

What are the 4 diseases of dense granule storage pool deficiency?

A
  1. Hermansky Pudiak
  2. Chediak-Higashi
  3. Thrombocytopenia with absent radii
  4. Wiskott-Aldrich
44
Q

Hermansky Pudiak presents with?

A

Severe oculocutaneous albinism

45
Q

Chediak Higashi presents with?

A

Partial albinism, large cytoplasmic granules, defective WBC function

46
Q

Defects in thromboxane synthesis occur due to?

A

Defects in the AA pathway

47
Q

What is an extrinsic acquired qualitative disorder?

A

Normal platelet but bad environment

48
Q

What are the 2 causes of extrinsic qualitative

A

Uremia and paraproteinemia where IgG coats the platelets

49
Q

What two things can cause intrinsic qualitative disorders?

A

Drugs and myeloproliferative neoplasms

50
Q

What is the most frequent inherited bleeding disorder?

A

von Willebrand disease

51
Q

How is vWF stored?

A

Weibel-palade bodies in endothelial cells and alpha granules of platelets

52
Q

What are the two general functions of vWF?

A

Adhesion and a carrier protein for factor 8

53
Q

What can exacerbate bleeding in those with vWF disease?

A

Aspirin

54
Q

What types of vWF disease lead to severe bleeding episodes?

A

2 and 3

55
Q

How much deficiency is there in vWF type 1? Leads to mild/moderate/severe disease?

A

Partial deficiency with normal vWF, mild to moderate disease

56
Q

Type 1 or 3 makes up the majority?

A

Type 1

57
Q

Describe vWF disease type 3.

A

Severe disorder, total or near deficiency of vWF, secondary deficiency of factor 8 and defect in clotting

Systemic Pathology Test 2 (10 decks)

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