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
Q

mucus membrane bleeding

A

epistaxis
gingival
vaginal bleeding
petechiae, purpura

26
Q

von willebrand disease : qualitative alterations in the vWF structure and function

A

Type 2

27
Q

von willebrand disease : least common and most severe

A

type 3

28
Q
  • complete absence of vWF in plasma or storage organelle
  • autosomal recessive
A

type 3 - vWD

29
Q

list of acquired vWD

A
  • lymphoproliferative disease
  • tumors
  • autoimmune disease
  • cardiac/vascular disease
  • medications (valproic acid)
  • hypothyroidism
30
Q

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.

A

EDTA ; NA citrate

31
Q

for TTP this rare disorder of the ______ system will cause patient to require _____ to remove inhibitors of ____ factor.

A

anticoagulation ; plasmapheresis ; VW

32
Q

give pt ______ to replace normal VW factor

A

FFP (fresh frozen plasma)

33
Q

this is a rare but serious disease that requires care under hematologist

A

TTP

34
Q

Giant Platelets (abnormalities)

A
  • May-Hegglin Anomaly (giant platelets and Dohle Bodies in the neutrophils)
  • Bernard Soulier syndrome
  • Alport syndrome
  • Storage pool syndrome
35
Q
  • normal total platelet mass
  • normal platelet number in circulation
A

pseudothrombocytopenia

36
Q

pseudothrombocytopenia causes

A
  • inadequate anticoagulated blood sample
  • EDTA-induced platelet clumping
  • Giant platelet
  • Platelet satellitism
37
Q

associated with decreased or ineffective magakaryopoiesis and thrombopoiesis

A

decreased platelet production

38
Q

decreased platelet production marrow damage

A
  • aplastic anemia
  • fanconi’s anemia (defect in DNA repair genes)
  • malignancy (w/ or w/o myelopthisis)
39
Q

decreased platelet production is also associated with

A
  • congenital defect (Congenital thrombocytopenias)
  • PNH
40
Q

decreased platelet production viral infections

A

rubella, CMV, EBV, HIV, Hep-C

41
Q

decreased platelet production ineffective production

A

Nutritional Deficiencies: B12, Folate, Severe Fe deficiency

42
Q

decreased platelet production drugs

A

thiazides, estrogen, chemotherapy, linezolid

43
Q

decreased platelet production toxins

A

alcohol, cocaine

44
Q

decreased platelet production marrow failure (pancytopenia)

A

aplastic anemia, chemotherapy, toxins

45
Q

decreased platelet production is also associated with

A

B12, folate or iron deficiency
viral infection

46
Q

decreased platelet production : drugs that can selectively reduce platelet production

A
  • alcohol
  • estrogens
  • thiazides
  • chlorpropamide
  • interferon
47
Q

decreased platelet production : amegakaryocytic thrombocytopenia

A
  • myelodysplasia (pre-leukemia)
  • immune (related to aplastic anemia)
48
Q

decreased platelet production is also related to the ___ thrombocytopenia and ____ thrombocytopenia

A

cyclic thrombocytopenia (rare)
inherited thrombocytopenia

49
Q

TTP is characterized _______ anemia and profound _____

A

microangiopathic hemolytic anemia ; profound intravascular platelet clumping

50
Q

disease was first reported in 1923 at beth israel in nyc

A

TTP

51
Q

the 16 year old girl who presented with

A

anemia, petechiae, coma and death

52
Q

in first TTP, terminal arterioles and cappilaries were occluded by ______ mostly composed of platelets

A

hyaline thrombi

53
Q

treatment of platelet disorders - congenital disorders

A
  • platelet transfusions (leucodepleted, HLA compatible and irradiated)
  • DDAVP
  • Tranexamic acid
  • Recombinant factor VIIa
  • Bone marrow transplantation