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PLATELET DISORDERS Flashcards

1
Q

disorder of platelet adhesion - deficiency of glycoprotein Ib/Ix

A

bernard-soulier syndrome

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2
Q

disorders of platelet aggregation - deficiency of glycoprotein IIb/IIIa

A

Glanzmann-Thrombasthenia

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3
Q

alpha or dense granules deficiency

A

disorders of platelet secretion

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4
Q

platelets fail to promote activation of the blood clotting proteins

A

disorders of platelet procoagulant activity

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5
Q

drugs like aspirin, non-steroidal anti-inflammatory drugs like indomethacin, ibuprofen

A

acquired platelet function disorders

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6
Q

Defect in adherence - The glycoprotein (Ib) receptor for VIII:Vwf is absent on platelet membrane and the Rx: platelets transfusion

A

Bernard-Soulier Syndrome

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7
Q

Defect in aggregation - the platelets lack surface glycoprotein receptor (IIb, IIIa) necessary for binding fibrinogen (fibrinogen receptor) and bleeding is prolonged

A

Glanzmann Thrombasthenia

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8
Q

congenital abnormalities of platelets can be divided into ________ and those of _______

A

disorders of platelet production ; platelet function

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9
Q

congenital abnormalities all are very ____

A

rare

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10
Q

congenital abnormal in general, cause moderate to severe _______

A

severe problems

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11
Q

autosomal recessive preleukemic condition which often presents as thrombocytopenia with skeletal or genitourinary abnormalities

A

Fanconi’s Anemia

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12
Q

congenital abnormalities laboratory features

A
  • abnormal chromosomal fragility
  • the condition can be cured with bone marrow transplantation (BMT)
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13
Q

thrombocytopenia clinical symptoms typically not seen until ______

A

<100 x 10^9/L

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14
Q

thrombocytopenia more often _____

A

<50 x 10^9/L

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15
Q

thrombocytopenia spontaneous bleeding occurs ____

A

<20 x 10^9 / L

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16
Q

thrombocytopenia life-threatening _______

A

<10 x 10^9/L

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17
Q

thrombocytopenia clinical manifestation

A
  • petechiae
  • menorrhagia
  • spontaneous bruising
  • fatal bleeding into the CNS may occur
  • spontaneous bleeding in GI-tract, GU-tract and nose
  • prolonged BT but normal coagulation tests
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18
Q

thrombocytopenia is due to

A
  1. deficient platelet production
  2. abnormal platelet distribution (splenic sequestration)
  3. increased destruction
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19
Q
  • secondary to an underlying disease
  • most common cause of excess or abnormal bleeding
A

thrombocytopenia

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20
Q

thrombocytopenia platelet count

A

< 100,000 cu mm

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21
Q

platelet count <50, 000

A

variable risk

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22
Q

platelet count <20,000

A

transfusion appropriate

23
Q

platelet count <10,000

A

spontaneous hemorrhage

24
Q

platelet dysfunction

A

mucus membrane bleeding

25
mucus membrane bleeding
epistaxis gingival vaginal bleeding petechiae, purpura
26
von willebrand disease : qualitative alterations in the vWF structure and function
Type 2
27
von willebrand disease : least common and most severe
type 3
28
- complete absence of vWF in plasma or storage organelle - autosomal recessive
type 3 - vWD
29
list of acquired vWD
- lymphoproliferative disease - tumors - autoimmune disease - cardiac/vascular disease - medications (valproic acid) - hypothyroidism
30
thrombocytopenia lab error: can have platelet clumping due to _____ tube this may cause platelet count to appear false low - peripheral smear examination can confirm normal platelet count. BLood can be collected in ____ tube and re-run to confirm normal count.
EDTA ; NA citrate
31
for TTP this rare disorder of the ______ system will cause patient to require _____ to remove inhibitors of ____ factor.
anticoagulation ; plasmapheresis ; VW
32
give pt ______ to replace normal VW factor
FFP (fresh frozen plasma)
33
this is a rare but serious disease that requires care under hematologist
TTP
34
Giant Platelets (abnormalities)
- May-Hegglin Anomaly (giant platelets and Dohle Bodies in the neutrophils) - Bernard Soulier syndrome - Alport syndrome - Storage pool syndrome
35
- normal total platelet mass - normal platelet number in circulation
pseudothrombocytopenia
36
pseudothrombocytopenia causes
- inadequate anticoagulated blood sample - EDTA-induced platelet clumping - Giant platelet - Platelet satellitism
37
associated with decreased or ineffective magakaryopoiesis and thrombopoiesis
decreased platelet production
38
decreased platelet production marrow damage
- aplastic anemia - fanconi's anemia (defect in DNA repair genes) - malignancy (w/ or w/o myelopthisis)
39
decreased platelet production is also associated with
- congenital defect (Congenital thrombocytopenias) - PNH
40
decreased platelet production viral infections
rubella, CMV, EBV, HIV, Hep-C
41
decreased platelet production ineffective production
Nutritional Deficiencies: B12, Folate, Severe Fe deficiency
42
decreased platelet production drugs
thiazides, estrogen, chemotherapy, linezolid
43
decreased platelet production toxins
alcohol, cocaine
44
decreased platelet production marrow failure (pancytopenia)
aplastic anemia, chemotherapy, toxins
45
decreased platelet production is also associated with
B12, folate or iron deficiency viral infection
46
decreased platelet production : drugs that can selectively reduce platelet production
- alcohol - estrogens - thiazides - chlorpropamide - interferon
47
decreased platelet production : amegakaryocytic thrombocytopenia
- myelodysplasia (pre-leukemia) - immune (related to aplastic anemia)
48
decreased platelet production is also related to the ___ thrombocytopenia and ____ thrombocytopenia
cyclic thrombocytopenia (rare) inherited thrombocytopenia
49
TTP is characterized _______ anemia and profound _____
microangiopathic hemolytic anemia ; profound intravascular platelet clumping
50
disease was first reported in 1923 at beth israel in nyc
TTP
51
the 16 year old girl who presented with
anemia, petechiae, coma and death
52
in first TTP, terminal arterioles and cappilaries were occluded by ______ mostly composed of platelets
hyaline thrombi
53
treatment of platelet disorders - congenital disorders
- platelet transfusions (leucodepleted, HLA compatible and irradiated) - DDAVP - Tranexamic acid - Recombinant factor VIIa - Bone marrow transplantation

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