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HEMATOLOGY > Platelet Disorders > Flashcards

Platelet Disorders Flashcards

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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
Patient with mutation in platelet growth factor receptor would present with...
No megakaryocytes- Congenital Amegakaryocytic Thrombocytopenia
26
How is May hegglin anomaly inherited
autosomal dominant- but may not know about it (mild to moderate bruising)
27
how is congenital amegakaryocytic thrombocytopenia inherited
autosomal recessive
28
How is Wiskott aldrich syndrome inherited?
X linked recessive
29
Tx for Wiskott aldrich syndrome
No NSAIDS, splenectomy (monitor, vaccinate frequently), platelets
30
Tx for Congenital Amegakaryocytic Thrombocytopenia
Bone marrow transplant (done in utero for high risk fetus because of the trauma associated with birth)
31
When should Neumega be initated after chemo treatment?
6-24 hours after, because it takes 3 days for it to work
32
What are non-inherited reasons you would see a decrease in platelet production?
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
Patient on tolbutamide for Type II diabetes may see onset of thrombocytopenia in.... next mode of action?
2-8 weeks after starting medication. Stop drug and give something else- recovery will take 7-10 days (rmbr life span of platelets)
34
You prescribe thiazide diuretics to HTN patient. What side effect are you careful to monitor during F/U in 4-6 weeks?
Thrombocytopenia
35
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?
Possibly ITP
36
When should you initiate tx for ITP?
when platelet count gets less than 20-30,000 or if bleeding. 30-50,000/ul count is ok and usually stable
37
Risk associated with WinRho administration in treatment of ITP?
Hemolytic anemia
38
Goal in pregnancy associated ITP
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
Deficiency in ADAMST13 causes...
Inherited or acquired (immune) TTP
40
Pentad of symptoms in thrombotic microangiopathies
Fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurologic deficits (TTP), renal failure (Hemolytic Uremic Syndrome)
41
TTP and ITP may both be caused by secondary causes such as
HIV, SLE, CLL
42
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?
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
Tx for TTP
if inherited- plasmapheresis, FFP. If immune-mediated, prednisone.
44
Tx for Hemolytic Uremic syndrome
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
Classic symptoms from HIT
Thrombocytopenia occuring 4-5 days after administration of heparin, thrombosis, and skin reactions
46
Antidote for HIT
1 mg protamine sulfate IV for every 100 IU of active heparin
47
2 reasons why you would administer heparin
Prophylactic dosing if someone at risk of getting DVT or for management/treatment of venous thrombosis
48
Prophylactic dosing for UFH
5000 IU subq 2 hours before surgery, and 5,000 IU subq every 8-12 hours after surgery
49
UFH tx for venous thrombosis
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
LMWH prophylactic dosing
Enoxaparin 30 mg SC BID or 40 mg once a day for 7-10 days
51
LMWH tx for venous thrombosis
ENOXAPARIN 1 mg/kg BID or 1.5 mg/kg daily
52
Name 2 direct thrombin inhibitors used to replace heparin in people that have HIT
Argatroban and Lepirudin
53
In ppl with HIT, initiate DTI first to get platelet levels up. Next step?
Get platelet count up to 100,000 then add coumadin. Discontinue DTI when coumadin is therapeutic
54
What is Enoxaparin
LMWH treatment for venous thrombosis and prophyactic dosing tx
55
What causes destruction of platelets that are not immune mediated?
Inherited TTP, Hemolytic Uremic Syndrome, infection, mechanical valves, DIC, Pre-eclampsia, HELLP syndrome
56
pregnancy associated bleeding disorders causing thrombocytopenia
Pregnancy associated ITP, pre-eclampsia, and HELLP syndrome
57
HELLP syndrome associated with pregnancy in what trimester
Third
58
How many patients experience thrombosis in HIT?
50% of patients in first month
59
Tx for Bernard Soulier syndrome and Glandzamann thrombasthenia
Transfusions of normal platelets because functional problem
60
How is bernard soulier inherited
Autosomal recessive deficiency of GpIb, but autosomal dominant form has also been identified
61
How is Glandzmann thrombasthenia
autosomal recessive, deficiency of GpIIb/IIIa
62
most common inherited bleeding disorder- prevalence
VonWillebrand Disease, Type I. 1% of all people have it, but only 10% are symptomatic
63
How is vW disease inherited?
Autosomal dominant in Types I, IIa, and IIb. Type III is autosomal recessive
64
In which inherited bleeding disorder will you see menorrhagia?
vonWillebrand disease
65
Type I vW disease
decrease in VWf causing functional defects of platelets
66
Type II vW disease
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
Type III vW disease
vWf nearly absent, autosomal recessive
68
If need to replenish someone with clotting factors plus fibrinogen, what to give?
Cryoprecipitate- higher levels of fibrinogen compared to FFP
69
Tx for vonwillebrand disease
DDAVP, FFP/cryoprecipitate, oral contraceptives, Vwf and Factor VIII
70
Diagnosis of Vonwillebrand disease
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
connection between vWf and Factor VIII
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
More severe granule disorder
Dense granule deficiency - mild to moderate bleeding, compared to grey platelet syndrome- life long mild bleeding
73
What disorder has absence of alpha granules
Grey platelet syndrome
74
tx of granule disorders
Platelet transfusions

HEMATOLOGY (5 decks)

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