Platelet Disorders Flashcards

1
Q

*What is the function of thrombopietin?

A

Regulate platelet production

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2
Q

What is the normal count of platelets?

A

150, 000-450, 000

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3
Q
  • What is the normal life span of a platelet?
A

7-10 days

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4
Q
  • Where are platelets produced?
A

In the liver and kidneys

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5
Q
  • What amount of platelets is normally trapped in the spleen?
A

1/3 of platelet mass

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6
Q
  • Describe the composition of platelets
A

Granules, membrane glycoproteins, membrane receptors

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7
Q
  • What is the function of platelets?
A

To form a mechanical plug during normal hemostatic response to vascular injury

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8
Q

What are the 4 steps involved in platelet function?

A

Adhesion, activation, aggregation, and interaction with coagulation factors

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9
Q

What test is used to test the amount of platelets?

A

CBC

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10
Q

What is the most common cause of bleeding?

A

Platelet disorders

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11
Q
  • Define thrombocytopenia
A

Decreased number of platelets or defective platelet function

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12
Q

What are the causes of thrombocytopenia?

A
  • Underproduction of platelets

- Increased rate of platelet removal from circulation

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13
Q
  • What are the clinical characterics of thrombocytopenia?
A
-Mucocutaneous bleeding (petechiae,
confluent, purpura, ecchymosis)
-Nose and/or gum bleeding
-Heavy menstrual bleeding in women
-Excessive bleeding after minor cute
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14
Q

*At which level should urgent platelet transfusion occur?

A

When platelet count < 10,000/L (regardless of clinical bleeding)

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15
Q

*At which level should platelet transfusion occur due to traumatic bleeding?

A

When platelet count is less than 50,000/L

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16
Q
  • At which level should platelet transfusion occur due to clinical or spontaneous bleeding?
A

When the platelet count is less than 10,000 to 20,000/L.

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17
Q

*Define Immunothromboytopenia (ITP)

A

Autoimmune disorder characterized by platelets bound antibodies

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18
Q

*What is the etiology of Immunothrombocytopenia (ITP)?

A

Idiopathic

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19
Q
  • Describe the diagnostic evaluation if ITP.
A

Diagnosis of exclusion: rule out all causes of thrombocytopenia; platelet antibody is non specific

20
Q
  • Describe treatment of ITP
A
  • Steroids (mainly)
  • IVIG
  • Rituximab (antibod
  • Splenectomy

-No treatment if platelet count >20K and non clinical hemorrhages

21
Q
  • What s/sx are associated with Thrombocytic thrombocytopenic purpura (TTP)?
A
  • Classic “pentad” (40%)
  • Fever
  • Neurological symptoms
  • Renal insufficiency
  • Thrombocytopenia (petechiae)
  • MAHA (hemolytic anemia)
22
Q
  • What treatment is used for thrombocytic thrombocytopenis putpura (TTP)?
A
  • Plasma exchange (main)
  • Rituximab
  • Corticosteroid
23
Q

*Define Hemolytic uremic syndrome (HUS)

A

a TMA that is characterized by the triad of hemolytic anemia (MAHA) , peripheral thrombocytopenia and renal failure in almost 100% of cases

24
Q
  • What s/sx present in HUS?
A
  • Diarrhea 2-12 days & later b/C bloody 1-2 days after

- Intense Abd pain and painful defecation

25
Q
  • What is the etiology of HUS?
A

Intestingal infection with Siga-toxin producing bacteria

  • Ecoli 0157: H7
  • Shigella dysenteriae serotype 1
26
Q
  • What treatment is used for typical HUS?
A

(symptomatic) : Hemodialysis, BP control RX, fluid mgnt

- No NSAIDS, antibiotics, or narcotics

27
Q
  • What treatment is used for atypical HUS?
A
  • Plasma exchange

- Eculizumab

28
Q

What is Disseminated intravascular coagulopathy (DIC)

A

Pathological imbalance in the procoagulant and anticoagulant systems that lead to unregulated thrombin generation

29
Q
  • What is the treatment of DIC?
A

TREAT UNDERLYING DISEASE

  • Clotting factor and inhibitor replacement
  • Pharmacologic Inhibitors
30
Q

*What are the characteristics of HELLP syndrome?

A

Hemolysis, Elevated Liver enzymes, and Low Platelets

31
Q

*What is the treatment for HELLP syndrome?

A

Fetal extraction/delivery

32
Q
  • What is the function of the spleen?
A
  • to remove particulates from the blood stream
  • Remove senescent/deformed RBCs from circulation
  • Trap and destroy antibody cotatefd platelets or RBC in pts with autoimmune disorders
33
Q
  • What percentage of platelets can reside in the spleenwith enlargement.
A

up to 90% in cases of extreme splenomegaly

34
Q
  • Define hyperspleenism
A

disorder which causes the spleen to rapidly and prematurely destroy blood cells.

35
Q

*Define splenomegaly

A

Abnormal enlargement of the spleen

36
Q

*What affect does Cardiopulmonary Bypass/ECMO have on platelets?

A

Platelets ACTIVATED by contact with ARTIFICIAL SURFACES during surgery

37
Q
  • What effect does Uremia have on platelets
A

Multifactorial including vascular abnormalities, anemia, and circulating metabolites cause defects in platelet function and adhesion

38
Q
  • What effect does myelopliferative disorders (MPD) have on platelets?
A
  • Clonal abnormalities in megakaryocytes produces defective platelets
  • Patient may present with hemorrhage or thrombosis
39
Q

*What two congenital disorders of platelet formation cause a defect in platelet-vessel interaction?

A
  • Von Willebrand disease

- Bernard-Soulier syndrom

40
Q
  • What defect does

Glanzmann Thrombasthenia have on platelets?

A

Causes defects in platelet-platelet interaction

41
Q

What is the treatment for Glanzman thrombasthenia?

A
  • Platelet transfusion (mainly)
  • rFVIIa if platelet antibodies develop
  • Bone marrow transplant
42
Q

What is the normal MPV volume?

A

8-11

43
Q

What disease presents with giant platelets?

A

Bernard-Soulier syndrome

44
Q

What defect is present in Bernard-Soulier syndrome?

A

Defect in platelet membrane glycoprotein IB-IX (VWF receptor) which prevents platelets from adhering to BV

45
Q

Which disorder presents with neutophil cytoplasmic inclusions (Dohle bodies)?

A

May-Hegglin Syndrome

46
Q

What disorder presents with hypogranular platelets (absence of platelets)

A

Gray Platelet syndrome

47
Q

Define thrombocytosis

A

platelet count greater than 450,000/microL