3.3b RBC: Bleeding Disorders Flashcards

0
Q

Functions of platelets (3)

A

Adhesion
Aggregation
Degranulation/Release Reaction

Note: any abnormality in those three will affect bleeding time

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1
Q

What labs determine PLATELET NUMBER and FUNCTION

A

Bleeding Time

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2
Q

Bleeding disorders (5)

A
  1. Vessel wall abnormalities
  2. Abnormalities in platelet number
  3. Abnormalities in platelet function
  4. Abnormalities in clotting factors
  5. Disseminated Intravascular Coagulation
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3
Q

In clotting factor dysfunction, what factors are involved and what lab parameter is affect?

A

“WAR-EXPAT”

Warfarin/Coumadin - Extrinsic factors - Affect PT

Other Mnemonics:

  1. PWET: PT is for Warfarin and Extrinsic pathway
  2. PHITT: PTT is for Heparin and Intrinsic pathway
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4
Q
  1. This involves small hemorrhages in the skin or mucous membranes
  2. Non-thrombocytopenic purpura (no decrease in platelets)
  3. All bleeding parameters are normal
A

Vessel Wall Abnormalities

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5
Q

What are the causes of vessel wall abnormalities? (5)

A
  1. Infection with rashes
    - Meningococcemia
    - Measles
    - Infective endocarditis
  2. Decreased collagen
    - Scurvy
    - Ehlers Danlos
  3. Drugs
    - PCN
    - Sulfa drugs
  4. Henoch Schonlein Purpura
    - Immune complexes circulating in blood attack blood vessels
  5. Hemorrhagic Telangiectasia
    - aka Weber-Osler-Rendu Syndrome
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6
Q

Bleeding disorder involving hemorrhages in skin and mucous membranes, GI, GU, easy bruisability

A

Abnormalities in platelet number (thrombocytopenia)

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7
Q

One of the complications of thrombocytopenia

A

Intracranial bleeding (usually the cause of death in adults)

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8
Q

What is the picture of PT and PTT in bleeding that results from thrombocytopenia?

A

Normal

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9
Q

What are the causes of Abnormalities in platelet number? (5)

A
  1. BM disease (decreased platelet production)
  2. Infective megakaryopoiesis (B12 deficiency)
  3. Increased destruction (decreased platelet survivial)
  4. Massive transfusion (plasma volume and red cell mass are reconstituted but number of circulating platelets is reduced)
  5. Sequestration (thrombocytopenia may develop in patients with splenomegaly)
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10
Q

What are examples of BM diseases that decreased platelet production? (2)

A
  1. Aplastic anemia

2. Myelopthisic anemia

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11
Q

Infective megakaryopoiesis results from deficiency in?

A

B12

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12
Q

What are the types of Heparin induced thrombocytopenia? (2)

A
  1. Type I HIT

2. Type II HIT

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13
Q

The ff describe which type of Heparin Induced Thrombocytopenia (HIT)?

  1. Thrombocytopenia develops 1-2 DAYS after heparin initiation
  2. CONTINUE to give heparin
  3. Mechanism of thrombocytopenia is NON-IMMUNE (it’s due to a DIRECT EFFECT of HEPARIN on platelet activation)
A

Type I HIT

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14
Q

The ff describe which type of Heparin Induced Thrombocytopenia (HIT)?

  1. Thrombocytopenia occurs 1-2 WEEKS
  2. IMMUNE-MEDIATED (body develops Ab against platelets) :. must STOP heparin because it will continue to destroy platelets –> bleeding
A

Type II HIT

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15
Q

The ff describes which cause of thrombocytopenia?

  1. Plasma volume and red cell mass are reconstituted but number of circulating platelets is reduced
  2. Throbocytopenia occurs because whole/packed blood that comes from the fridge ha no functional platelets
  3. Produces DILUTIONAL THROMBOCYTOPENIA
A

Massive Transfusion

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16
Q

What diseases are associated with abnormalities in platelet number? (2)

A
  1. Idiopathic Thrombocytopenic Purpura (ITP)

2. Thrombotic Thrombocytopenic Purpura (TTP)

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17
Q

The ff describes which disease associated which abnormality in platelet number?

  1. Autoimmune disease: Ab vs. platelets
  2. BM: normal or with increased megakaryocytes
  3. Steroids are given
  4. Platelet concentrates are a contraindication
A

Idiopathic Thrombocytopenic Purpura (ITP)

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18
Q

What are the types of ITP? (2)

A
  1. Acute ITP

2. Chronic ITP

19
Q

Which type of ITP is being described?
1. Caused by autoAb against platelets
2. A self-limited disorder in CHILDREN following viral infection for 1-2weeks
3, Usually resolves spontaneously within 6 months

A

Acute ITP

20
Q

Which type of ITP is being described?

  1. Caused by autoAb against platelets (specifically, their membrane glycoproteins: IIb-IIIa or Ib-IX)
  2. In majority of cases, anti-platelet Ab seen is IgG
  3. Seen in ADULTS, especially reproductive females with autoimmune disorders or lymphoproliferative disease
  4. An alternative treatment to steroids is removal of spleen
A

Chronic ITP

21
Q

What is the pathogenesis of Chronic ITP?

A

Antiplatelet Ab act as opsonins (recognized by IgG Fc receptors) –> increased platelet destruction

Like in autoimmune hemolytic anemia

22
Q

In TTP, autoAb are directed against which membrane glycoprotein?

A

IIb-IIIa or Ib-IX

23
Q

The ff describes which disease associated which abnormality in platelet number?
1. It is characterized by MICROANGIOPATHIC hemolytic anemia, fever, transient neurologic deficits, renal failure, thrombocytopenia, usually in the 4th decade of life

A

TTP

24
Q

What is the morphology seen in chronic TTP

A

Widespread hyaline membrane microthrombi

25
Q

What is the pathogenesis in TTP?

A

Excessive activation of platelets (due to deficiency of enzyme)

26
Q

Which enzyme is deficient in TTP?

A

ADAMTS13/vWF protease

27
Q

What happens when there is deficiency in ADAMTS13/vWF protease?

A

Accumulation of vWF in blood vessel –> stimulates adhesion, aggregation, thrombosis

28
Q

What are the functions of vWF? (2)

A
  1. Protects Factor VIII

2. Helps in platelet adhesion

29
Q

What is the end results of TTP?

A

Microthrombi in different parts of the body

30
Q

Treatment of TTP

A

Plasma exchange

31
Q

What is a differential diagnosis of TTP?

A

Hemolytic Uremic Syndrome (HUS)

32
Q

What similarity (1) and difference (1) does TTP and HUS have?

A

Similar: Microthrombi everywhere

Difference lies in etiology

  • (HUS) E.coli secondary to gastroenteric effect –> Shigella-like toxin that will cause thrombosis
  • (HUS) No neurologic deficits
  • (HUS) Medical emergency that needs immediate plasma transfusion
33
Q

Examples of INBORN abnormalities in platelet function (3)

A
  1. Von Willebrand Disease
  2. Bernard Soulier Disease
    3 Glanzmann Thrombasthenia
34
Q

Pathogenesis of Von Willebrand Disease

A

Defective ADHESION due to deficient vWF (needed for platelet function and Factor VIII stability)

35
Q

The ff are s/sx of which inborn abnormality of platelet function?

  1. Spontaneous bleeding from mucous membrane
  2. Excessive bleeding from wounds
  3. Menorrhagia
  4. Hemarthrosis
A

Von Willebrand Disease

36
Q

What would the ff lab parameters look like in Von Willebrand Disease?

  1. BT
  2. aPTT
  3. Ristocetin Aggregation Test
A
  1. Increased BT
  2. Increased aPTT
  3. Reduced rostocetin aggregation test
  4. NORMAL PLATELET COUNT
37
Q

In Bernard Soulier Disease, there is defective adhesion due to absence of?

A

Glycoprotein Ib

38
Q

In Glanzmann Thrombasthenia, there is deficient AGGREGATION due to the absence of? (2)

A
  1. Glycoprotein IIb and IIIa
39
Q

Acquired abnormalities in platelet function may be caused by? (2)

A
  1. Aspirin/NSAIDs (inhibit COX, suppresses PG)

2. Uremia

40
Q

Deficiency in which factors result to abnormalities of clotting factors? (3)

A
  1. Factor VIII - Hemophilia
  2. Factor IX - Christmas disease/Hemophilia B
  3. Factor II, VII, IX, X - Liver disease/Vitamin K Deficiency
41
Q

In abnormalities in clotting factors, what do the ff labs look like?

  1. Platelet count
  2. BT
  3. CT
  4. PT
  5. aPTT
A
  1. Normal platelet count
  2. Normal BT
  3. Increased CT
  4. Increased PT
    (involves extrinsic and common factors: Factors VII, I, II, V, X)
  5. Increased aPTT
    (involves extrinsic and common factors)
42
Q

It is an acute, subacute, or chronic thrombo-hemorrhagic disorder occurring as a complication in a variety of diseases

aka Consumption Coagulopathy

A

Disseminated Intravascular Coagulation (DIC)

43
Q

What is the morphology seen in DIC?

A

Microthrombi

44
Q

What is the lab diagnosis in Acute DIC?

A

All parameters are ABNORMAL:

  1. Decreased platelet
  2. Increase in everything else
45
Q

What is the lab diagnosis in Chronic DIC?

A

(+) D-dimers and fibrin split products

46
Q

What are the clinical syndromes associated with bleeding? (2)

A
  1. Waterhouse-Friedrichsen Syndrome
    - Meningococcemia –> Adrenal hemorrhage
  2. Sheehan’s Postpartum Necrosis
    - Pituitary necrosis after complicated delivery