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MT2 > HEMA 2 - PLATELET DISORDERS > Flashcards

HEMA 2 - PLATELET DISORDERS Flashcards

1
Q

platelets are produced predominantly by the ______ as a result of budding of the _____

A

Bone Marrow Megakaryocytes ; Cytoplasmic Membrane

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2
Q

Megakaryocytes are derived from the _______, which is stimulated to differentiate to mature megakaryocytes under the influence of various cytokines including ___________.

A

Hemopoietic Stem Cells ; Thrombopoietin

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3
Q

disorders of platelet adhesion - deficiency of glycoprotein Ib/IX

A

bernard-soulier syndrome

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4
Q

disorders of platelet aggregation - deficiency of glycoprotein IIb/IIIa

A

glanzmann-thrombasthenia

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5
Q

disorders of platelet secretion

A

alpha or dense granules deficiency

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6
Q

platelets fail to promote activation of the blood clotting proteins

A

disorders of platelet procoagulant activity

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7
Q

acquired platelet function disorders, drug like …

A

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

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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

In general they cause moderate to severe bleeding problems.

A

Congenital abnormalities

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11
Q
  • an 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 lab features

A
  • Abnormal chromosomal fragility.
  • The condition can be cured with bone marrow transplantation (BMT)
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13
Q

rule out pseudotrhombocytopenia

A
  • sequestration
  • production
  • destruction
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14
Q

look for splenomegaly

A

sequestration

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15
Q

causes of splenomegaly (sequestration)

A
  • infection
  • inflammation
  • congestion
  • malignancy
  • red cell disorders
  • storage disorders
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16
Q

bone marrow investigation review meds

A

production

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17
Q

included in production - bone marrow investigation review meds

A
  • aplasia
  • infiltration
  • ineffective megakaryopoiesis (e.g MDS)
  • selective impairment of platelet production
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18
Q

look for underlying disorders review meds

A

destruction

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19
Q

included in destruction - bone marrow investigation review meds

A
  • immune auto-immune (ITP, SLE) rugs infection allo-immune
  • none-immune seosis DIC, TTP, HUs hypertensive disorders of pregnancy
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20
Q

also included in approach to thrombocytopenia

A

artefactual (false) or pseudothrombocytopenia and congenital thrombocytopenia

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21
Q

clot in the sample and platelets clumped

A

artefactual (false) or pseudothrombocytopenia

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22
Q

rare inherited disorders (e.g may hegglin anomaly)

A

Congenital Thrombocytopenia

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23
Q

platelet count is less than 100,00 cu mm =

A

thrombocytopenia

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24
Q

Platelet count <50,000

A

variable risk

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25
Q

Platelet count <20,000

A

transfusion appropriate

26
Q

Platelet count <10,000

A

spontaneous hemorrhage

27
Q

mucus membrane bleeding =

A

platelet dysfunction

28
Q

platelet dysfunction includes

A

Epistaxis
Gingival
Vaginal Bleeding
Petechiae
Purpura

29
Q

a type of von Willebrand disease that is qualitative alterations in the vWF structure and function

30
Q

a type of von Willebrand disease that is least common and most severe

31
Q

complete absence of vWF in plasma or storage organelle is a type ___ vWD

32
Q

acquired vWD inclues

A
  • lymphoproliferative disease
  • tumors
  • autoimmune disease
  • cardiac/valvular disease
  • medications such as valproic acid
  • hypothyroidism
33
Q

platelet morphology: giant platelets includes

A
  • May Hegglin Anomaly
  • Bernard-Soulier Syndrome
  • Alport Syndrome
  • Storage Pool Syndrome
34
Q
  • normal total platelet mass
  • normal platelet number in circulation
A

Pseudothrombocytopenia

35
Q

causes of pseudothrombocytopenia

A
  • inadequate anticoagulated blood sample
  • EDTA-induced platelet clumping
  • Giant platelet
  • Platelet satellitism
36
Q

Associated with decreased or ineffective megakaryopoiesis and thrombopoiesis.

A

Decreased Platelet Production

37
Q

decreased platelet production marrow damage includes

A

o Aplastic Anemia
o Fanconi’s Anemia (defect in DNA repair genes)
o Malignancy (with or without myelophthisis)

38
Q

decreased platelet production also deals with

A
  • Congenital Defects (congenital thrombocytopenia)
  • Paroxysmal Nocturnal Hemoglobinuria (PNH)
39
Q

decreased platelet production also deals with viral infections such as

A

o Rubella, CMV, EBV, HIV & Hep-C

40
Q

decreased platelet production also deals with ineffective production such as

A

o Nutritional Deficiencies such as B12, Folate, Severe Fe deficiency.

41
Q

decreased platelet production also deals with drugs such as

A

o Thiazides, Estrogen, Chemotherapy, Linezolid

42
Q

decreased platelet production also deals with toxins such as

A

o Alcohol, Cocaine

43
Q

decreased platelet production also deals with marrow failure (pancytopenia) such as

A

o Aplastic Anemia, Chemotherapy, Toxins

44
Q

decreased platelet production also deals with B-12, Folate or _____ deficiency

45
Q

decreased platelet production also deals with drugs that can selectively reduce platelet production such as

A

o Alcohol
o Estrogens
o Thiazides
o Chlorpropamide
o Interferon

46
Q

decreased platelet production also deals with Amegakaryocytic Thrombocytopenia such as

A

o Myelodysplasia (pre-leukemia)
o Immune (related to aplastic anemia)

47
Q

decreased platelet production also deals with ______ and ________ thrombocytopenia

A
  • Cystic thrombocytopenia (rare)
  • Inherited thrombocytopenia
48
Q

TTP is a short term for

A

THROMBOTIC THROMBOCYTOPENIC PURPURA

49
Q

TTP is characterized by __________ and ________________.

A

microangiopathic hemolytic anemia ; profound intravascular platelet clumping

50
Q

Evaluation of suspected hereditary and acquired disorders of platelet function.

A

PLATELET AGGREGATION TESTING

51
Q

Platelets normally contain three major types of granules

A
  • alpha granules
  • dense granules
  • lambda granules
52
Q

granule that contains fibrinogen, PF4, Factor V, Von Willebrand Factor.

A

Alpha Granules

53
Q

granule that contains ADP or ATP, calcium and serotonin

A

Dense Granules

54
Q

a granule that is similar to lysosomes and contain several hydrolytic enzymes.

A

o Lambda Granules

55
Q

Hereditary platelet function disorders includes

A

o Rare defects of adhesion = Bernard Soulier Syndrome

o Rare defects of aggregation = Glanzmann Thrombasthenia

o More common defects of secretion = Alpha or Dense Granule Deficiency, Aspirin-like Defects, or Other primary defects.

56
Q

Bernard Soulier Syndrome is a rare defects of _____

57
Q

Glanzmann Thrombasthenia is a rare defects of _____

A

aggregation

58
Q

Alpha or Dense Granule Deficiency, Aspirin-like Defects, or Other primary defects is a more common defects of _______

59
Q

Acquired platelet function disorders are more common than the _________ and include _________.

A

hereditary disorders ; drug-induced platelet dysfunction

60
Q

drug-induced platelet dysfunction includes

A

o Aspirin
o NSAID’s
o Clopidogrel
o Antibiotics
o Various cardiovascular
o Psychotropic Drugs
o Uremia
o Myeloproliferative disorders

61
Q

TREATMENT OF PLATELET DISORDERS (Congenital Disorders)

A
  • Platelet Transfusion = Leucodepleted, HLA compatible and Irradiated
  • DDAVP
  • Tranexamic Acid
  • Recombinant factor VIIa
  • Bone Marrow Transplantation

MT2 (45 decks)

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