Platelets: Qualitative and Quantitative Disorders Flashcards

1
Q

3 functions of von Willebrand Factor (vWF)

A
  • Carrier protein for Factor VIII (VIII:C)
  • Participates in platelet’s adhesion to subendothelial collagen
  • Facilitates a normal aggregation response to ristocetin
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2
Q

von Willebrand disease

- Pathogenesis

A

Clinical disorder due to deficiency of vWF

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3
Q

von Willebrand disease

- Bleeding time results

A

Increased

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4
Q

von Willebrand disease

- Closure time results (PFA)

A

Increased

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5
Q

von Willebrand disease

- Platelet adhesion result

A

Abnormal

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6
Q

von Willebrand disease

- Platelet aggregation w/ ristocetin

A

Abnormal ristocetin induced aggregation

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7
Q

von Willebrand disease

- Platelet count

A

Can be normal or abnormal → not a hallmark

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8
Q

von Willebrand disease

- APTT result

A

Prolonged due to ↓ VIII:C

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9
Q

Bernard-Soulier syndrome

- Pathogenesis

A

Missing GPIb/IX receptor complex

- Platelets cannot adhere to subendothelium via vWF

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10
Q

Bernard-Soulier syndrome

- Bleeding time result

A

Prolonged

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11
Q

Bernard-Soulier syndrome

- Platelet aggregation w/ ADP and ristocetin

A
  • ADP: normal

- Ristocetin: abnormal

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12
Q

Bernard-Soulier syndrome

- Platelet adhesion result

A

Abnormal

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13
Q

Bernard-Soulier syndrome

- Platelet count

A

Decreased

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14
Q

Bernard-Soulier syndrome

- Platelet morphology

A

Giant platelets

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15
Q

Glanzmann’s thrombasthenia

- Pathogenesis

A

Missing GPIIb/IIIa receptor complex

- Platelets cannot bind to fibrinogen and bridge/attach to one another → cannot aggregate

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16
Q

Glanzmann’s thrombasthenia

- Bleeding time result

A

Prolonged

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17
Q

Glanzmann’s thrombasthenia

- Platelet adhesion result

A

Normal

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18
Q

Glanzmann’s thrombasthenia

- Platelet aggregation w/ ADP and ristocetin

A
  • ADP: abnormal

- Ristocetin: normal

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19
Q

Glanzmann’s thrombasthenia

- Platelet count

A

Normal

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20
Q

Glanzmann’s thrombasthenia

- Platelet morphology

A

Normal

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21
Q

5 acquired qualitative platelet disorders

A
  • Drug therapy (most common cause)
  • Renal disease (uremia)
  • Dysproteinemias
  • Liver disease
  • Platelet Abs
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22
Q

4 general causes for thrombocytopenia

A
  • ↓ platelet production
  • Ineffective thrombopoiesis
  • Abnormal distribution of platelets
  • ↑ destruction of platelets
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23
Q

Destruction occurs by processes other than the immune system (consumption, mechanical destruction)

A

Nonimmune-mediated platelet destruction

24
Q

Platelets become sensitized w/ Abs and RES, especially the spleen, destroys them

A

Immune-mediated platelet destruction

25
3 causes of nonimmune-mediated platelet destruction for thrombocytopenia
- Thrombotic thrombocytopenic purpura (TTP) - Hemolytic uremic syndrome (HUS) - Disseminated intravascular coagulation (DIC)
26
3 causes of immune-mediated platelet destruction for thrombocytopenia
- Idiopathic thrombocytopenic purpura (ITP) - Drug-induced → heparin-induced thrombocytopenia (HIT) - Neonatal alloimmune thrombocytopenia
27
``` Thrombocytopenic purpura (TTP) - Predisposing factors ```
- Infectious diseases - Autoimmune diseases - Drugs - Pregnancy - Hereditary
28
``` Thrombocytopenic purpura (TTP) - Clinical presentation ```
Characterized by - MAHA (jaundice, pallor, acute renal failure) - Thrombocytopenia - Neurologic dysfunction (headaches, seizures, vertigo, delirium) Mostly in adults
29
``` Thrombocytopenic purpura (TTP) - Platelet count ```
Decreased
30
``` Thrombocytopenic purpura (TTP) - Lab findings ```
- ↓ RBC, hemoglobin, hematocrit - ↑ reticulocytes - SCHISTOCYTES - Coag tests (PT, APTT) usually normal but it helps to distinguish from DIC
31
``` Thrombocytopenic purpura (TTP) - Type of anemia/mechanism ```
Hemolytic anemia??? Underlying disorder that damages vascular endothelium* - Caused by ultra high molecular weight vWF multimers present when endothelium-secreted vWF isn't properly proteolysed by its cleaving protease - Promotes deposition of platelet thrombi (platelet consumption, fragmented RBCs leading to intravascular hemolysis)
32
Hemolytic uremic syndrome (HUS) | - Predisposing factors
Acquired disorder that resembles TTP Characterized by - MAHA - Thrombocytopenia
33
Hemolytic uremic syndrome (HUS) | - Clinical presentation in contrast to TTP
- Usually no neurological manifestations - Intravascular clotting confined to the kidney leading to acute renal failure - Associated w/ bacterial toxins
34
Hemolytic uremic syndrome (HUS) | - Platelet count
Decreased
35
Hemolytic uremic syndrome (HUS) | - Lab findings
- Similar to TTP | - Additionally, abnormal urinalysis results: proteinuria, hematuria, RBC casts often
36
Hemolytic uremic syndrome (HUS) | - Clinical findings in children
- Classic HUS occurs in infants <2 yrs age | - Children present after a febrile illness with: vomiting, bloody diarrhea, MAHA leads to renal failure, hypertension
37
Hemolytic uremic syndrome (HUS) | - Clinical findings in adults
Link w/ malignancy and/or chemotherapy
38
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Age of onset ```
Childhood
39
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Previous infections? ```
Common
40
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Platelet count ```
Decreased (< 20,000/uL), so BM exam requested
41
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Bleeding characteristics ```
Abrupt onset of bruising, petechiae, and sometimes mucosal bleeding in a previously healthy patient
42
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Occurrence of spontaneous remission ```
Occurs in majority of patients
43
``` Acute idiopathic (immune) thrombocytopenic purpura (ITP) - Treatment ```
- Some patients do not require treatment due to spontaneous remissions - Severe causes require platelet transfusions and/or splenectomy (as spleen is primary site of platelet destruction)
44
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Age of onset ```
20-50 years old, females over males
45
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Previous infections? ```
Usually not associated
46
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Platelet count ```
Variable, 30,000-80,000/uL
47
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Bleeding characteristics ```
Insidious
48
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Occurrence of spontaneous remission ```
Relatively rare
49
``` Chronic idiopathic (immune) thrombocytopenic purpura (ITP) - Treatment ```
- Treat w/ steroids | - Refractory cases may require splenectomy and/or chemotherapeutic agents
50
Heparin-induced thrombocytopenia (HIT), type II, according to the basis for the possible thrombotic and hemorrhagic presentation
- In HIT, PF4 and heparin combine to form a surface complex against which the IgG antibody is directed - Platelets become activated, in vivo platelet aggregation with thrombosis is possible → thrombotic and hemorrhagic problems
51
An adverse effect of heparin therapy is called ____
Heparin-induced thrombocytopenia (HIT)
52
Develops when the mother lacks a specific platelet-specific Ag that the fetus has inherited from the father
Neonatal alloimmune thrombocytopenia
53
How do we get thrombocytopenia in neonatal?
Mother produces Abs to the fetal Ag which cross the placenta, and attach to the Ag-bearing fetal platelets, resulting in thrombocytopenia
54
This is autosomal dominant inheritance and giant dohle-like bodies in neutrophils, eosinophils, basophils, and monocytes are seen
May-Hegglin anomaly
55
4 clinical states in which thrombocytosis can be seen
- Hemorrhage or surgery - Inflammation - Exercise-induced - FDA
56
Describe thrombocytosis according to the etiology and the platelet count values
- Etiology: reactive phenomena--secondary to hemorrhage or surgery, inflammation, exercise-induced, FDA??? - Platelet count is ↑ but usually <1,000,000/cumm
57
Describe thrombocythemia according to the etiology and the platelet count values
- Etiology: MP disorder cause is unknown | - Platelet count exceeds 1,000,000/cumm