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HEMATOLOGY > Platelet Disorders > Flashcards

Platelet Disorders Flashcards

1
Q

Diagnostic plt count for elevated bleeding time

A

> 70,000/ul

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2
Q

Diagnostic plt count for spontaneous bleeding

A

> 20,000/ul

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3
Q

Diagnostic plt count for post-traumatic bleeding

A

20-50,000/ul

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4
Q

Causes of pseudothrombocytopenia

A

EDTA exposure, cold, and difficult venipuncture

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5
Q

Diameter of platelets

A

2-3micrometers

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6
Q

Agents dependent on GpIIb/IIIa in aggregation studies

A

epinephrine, ADP, and collagen

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7
Q

Desired platelet count to prevent spontaneous bleeding

A

Above 10-20,000/ul

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8
Q

Desired plt count before administration of Heparin (or other therapeutic anticoagulant)

A

Above 30-50,000/ul

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9
Q

Desired plt count before hip or knee replacement (MAJOR surgery)

A

Above 80-100,000/ul

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10
Q

Desired plt count before minor surgery (arthoscopy)

A

Above 50-80,000/ul

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11
Q

Desired plt count before insertion of central venous catheter

A

Above 20-50,000/ul

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12
Q

MOA cox inhibitors

A

Block production of thromboxane, preventing vasoconstriction and decreasing platelet aggregation

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13
Q

MOA ADP receptor inhibitors

A

Block ADP activation and recruitment of platelets

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14
Q

GpIIb/IIIa Inhibitors MOA

A

Block platelet aggregation by blocking GpIIb/IIIa

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15
Q

MOA Adenosine reuptake inhibitors

A

Increases cAMP which potentiates prostaglandin, which causes vasodilation and decreases platelet aggregation

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16
Q

ADP receptor inhibitors

A

CPT-PET
Clopidogrel-Plavix
Prasugrel- Effient
Ticlopidine-Ticlid

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17
Q

GpIIb/IIIa inhibitors

A

ARIETA
Abciximab-ReoPro
Eptifibatid-Integrelin
Tirofiban-Aggrastat

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18
Q

Adenosine reuptake inhibitor

A

Dipyradamole- Persantine

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19
Q

Manifestations in spontaneous bleeding

A

Bleeding on skin, from gums, from nose, mucous membranes (GI and genitourinary tracts), and intracranial bleeding

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20
Q

What is the number 1 cause of death in many of the platelet disorders?

A

Intracranial bleeding

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21
Q

What does May hegglin anomaly affect besides platelets

A

Affects WBC also- affects ability of WBC’s to mount a good immune response

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22
Q

5 year old with mild to moderate bruising after playing on playground one day, decreased and large platelets under microscope. Mother has a mutation in MYH9 gene. Diagnosis?

A

May-Hegglin Anomaly

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23
Q

6 month old boy presents with eczema and bloody diarrhea. IgM antibodies are low, and decreased platelets, platelets appear small under microscope. Diagnosis?

A

Wiskott-Aldrich Syndrome

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24
Q

Wiskott aldrich syndrome other name

A

Eczema thrombocytopenia immunodeficiency syndrome

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25
Q

Patient with mutation in platelet growth factor receptor would present with…

A

No megakaryocytes- Congenital Amegakaryocytic Thrombocytopenia

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26
Q

How is May hegglin anomaly inherited

A

autosomal dominant- but may not know about it (mild to moderate bruising)

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27
Q

how is congenital amegakaryocytic thrombocytopenia inherited

A

autosomal recessive

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28
Q

How is Wiskott aldrich syndrome inherited?

A

X linked recessive

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29
Q

Tx for Wiskott aldrich syndrome

A

No NSAIDS, splenectomy (monitor, vaccinate frequently), platelets

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30
Q

Tx for Congenital Amegakaryocytic Thrombocytopenia

A

Bone marrow transplant (done in utero for high risk fetus because of the trauma associated with birth)

31
Q

When should Neumega be initated after chemo treatment?

A

6-24 hours after, because it takes 3 days for it to work

32
Q

What are non-inherited reasons you would see a decrease in platelet production?

A

Radiation, chemo causing marrow aplaisia or hypoplasia, marrow infiltration because of malignancies or infections, ineffective megakaryopoiesis, and drug-induced from ethanol, DES, tolbutamide, or thiazide diuretics

33
Q

Patient on tolbutamide for Type II diabetes may see onset of thrombocytopenia in…. next mode of action?

A

2-8 weeks after starting medication. Stop drug and give something else- recovery will take 7-10 days (rmbr life span of platelets)

34
Q

You prescribe thiazide diuretics to HTN patient. What side effect are you careful to monitor during F/U in 4-6 weeks?

A

Thrombocytopenia

35
Q

Patient presents with easy bruising, petehiae, gum bleeding, nose bleeds. Order CBC- platelet count 20,000. Elevated LDH, bilurubin levels, decreased haptoglobin, anemia, enlarged platelets. Diagnosis?

A

Possibly ITP

36
Q

When should you initiate tx for ITP?

A

when platelet count gets less than 20-30,000 or if bleeding. 30-50,000/ul count is ok and usually stable

37
Q

Risk associated with WinRho administration in treatment of ITP?

A

Hemolytic anemia

38
Q

Goal in pregnancy associated ITP

A

If 1st trimester- keep plt count between 10-30,000. If second or third, keep it above 30,000. If C-section, keep it above 50,000.

39
Q

Deficiency in ADAMST13 causes…

A

Inherited or acquired (immune) TTP

40
Q

Pentad of symptoms in thrombotic microangiopathies

A

Fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurologic deficits (TTP), renal failure (Hemolytic Uremic Syndrome)

41
Q

TTP and ITP may both be caused by secondary causes such as

A

HIV, SLE, CLL

42
Q

Patient presents with petechiae, purpura, and pallor. You do CBC and platelet count is low- thrombocytopenia. Patient is SOB especially on exertion, pale, and fatigued. CBC shows reduced RBC count of 3 million/ul, so patient is anemic. WBC count is normal. Patient is also complaining of headaches, tingling, numbness, and confusion. What is the next step?

A

Suspect TTP. Get blood smear to check for schistocytes or bite cells and reticulocytosis. Increased bilirubin and LDH from ischemic tissue. Coag tests normal.

43
Q

Tx for TTP

A

if inherited- plasmapheresis, FFP. If immune-mediated, prednisone.

44
Q

Tx for Hemolytic Uremic syndrome

A

If children- self limiting, but have to be hospitalized with antibiotics.
In adults- often associated with SLE or other autoimmune disorders. Tx with plasmapheresis to get rid of any type of antibodies and FFP

45
Q

Classic symptoms from HIT

A

Thrombocytopenia occuring 4-5 days after administration of heparin, thrombosis, and skin reactions

46
Q

Antidote for HIT

A

1 mg protamine sulfate IV for every 100 IU of active heparin

47
Q

2 reasons why you would administer heparin

A

Prophylactic dosing if someone at risk of getting DVT or for management/treatment of venous thrombosis

48
Q

Prophylactic dosing for UFH

A

5000 IU subq 2 hours before surgery, and 5,000 IU subq every 8-12 hours after surgery

49
Q

UFH tx for venous thrombosis

A

5,000 units IV as bolus, followed by 1,000 units IV every hour after, OR 80 units/kg as bolus, followed by 18 units/hour after IV

50
Q

LMWH prophylactic dosing

A

Enoxaparin 30 mg SC BID or 40 mg once a day for 7-10 days

51
Q

LMWH tx for venous thrombosis

A

ENOXAPARIN 1 mg/kg BID or 1.5 mg/kg daily

52
Q

Name 2 direct thrombin inhibitors used to replace heparin in people that have HIT

A

Argatroban and Lepirudin

53
Q

In ppl with HIT, initiate DTI first to get platelet levels up. Next step?

A

Get platelet count up to 100,000 then add coumadin. Discontinue DTI when coumadin is therapeutic

54
Q

What is Enoxaparin

A

LMWH treatment for venous thrombosis and prophyactic dosing tx

55
Q

What causes destruction of platelets that are not immune mediated?

A

Inherited TTP, Hemolytic Uremic Syndrome, infection, mechanical valves, DIC, Pre-eclampsia, HELLP syndrome

56
Q

pregnancy associated bleeding disorders causing thrombocytopenia

A

Pregnancy associated ITP, pre-eclampsia, and HELLP syndrome

57
Q

HELLP syndrome associated with pregnancy in what trimester

A

Third

58
Q

How many patients experience thrombosis in HIT?

A

50% of patients in first month

59
Q

Tx for Bernard Soulier syndrome and Glandzamann thrombasthenia

A

Transfusions of normal platelets because functional problem

60
Q

How is bernard soulier inherited

A

Autosomal recessive deficiency of GpIb, but autosomal dominant form has also been identified

61
Q

How is Glandzmann thrombasthenia

A

autosomal recessive, deficiency of GpIIb/IIIa

62
Q

most common inherited bleeding disorder- prevalence

A

VonWillebrand Disease, Type I. 1% of all people have it, but only 10% are symptomatic

63
Q

How is vW disease inherited?

A

Autosomal dominant in Types I, IIa, and IIb. Type III is autosomal recessive

64
Q

In which inherited bleeding disorder will you see menorrhagia?

A

vonWillebrand disease

65
Q

Type I vW disease

A

decrease in VWf causing functional defects of platelets

66
Q

Type II vW disease

A

Type IIa- abnormality in protein that prevents multimer formation of vWf. Type IIb- abnormality in protein causing rapid clearance of the large multimeric forms

67
Q

Type III vW disease

A

vWf nearly absent, autosomal recessive

68
Q

If need to replenish someone with clotting factors plus fibrinogen, what to give?

A

Cryoprecipitate- higher levels of fibrinogen compared to FFP

69
Q

Tx for vonwillebrand disease

A

DDAVP, FFP/cryoprecipitate, oral contraceptives, Vwf and Factor VIII

70
Q

Diagnosis of Vonwillebrand disease

A

order CBC- normal platelet count. Bleeding test will be elevated because abnormal function. Platelet aggregation studies- ristocetin will be abnormal. PT normal, PTT abnormal, and decreased Factor VIII levels

71
Q

connection between vWf and Factor VIII

A

Vwf binds to Factor VIII in bloodstream. If levels of Vwf go down, Factor VIII gets cleared from body, so levels decrease in bloodstream leading to prolonged PTT

72
Q

More severe granule disorder

A

Dense granule deficiency - mild to moderate bleeding, compared to grey platelet syndrome- life long mild bleeding

73
Q

What disorder has absence of alpha granules

A

Grey platelet syndrome

74
Q

tx of granule disorders

A

Platelet transfusions

HEMATOLOGY (5 decks)

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