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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
Platelet count <20,000
transfusion appropriate
26
Platelet count <10,000
spontaneous hemorrhage
27
mucus membrane bleeding =
platelet dysfunction
28
platelet dysfunction includes
Epistaxis Gingival Vaginal Bleeding Petechiae Purpura
29
a type of von Willebrand disease that is qualitative alterations in the vWF structure and function
Type 2
30
a type of von Willebrand disease that is least common and most severe
Type 3
31
complete absence of vWF in plasma or storage organelle is a type ___ vWD
Type 3
32
acquired vWD inclues
- lymphoproliferative disease - tumors - autoimmune disease - cardiac/valvular disease - medications such as valproic acid - hypothyroidism
33
platelet morphology: giant platelets includes
- May Hegglin Anomaly - Bernard-Soulier Syndrome - Alport Syndrome - Storage Pool Syndrome
34
- normal total platelet mass - normal platelet number in circulation
Pseudothrombocytopenia
35
causes of pseudothrombocytopenia
- inadequate anticoagulated blood sample - EDTA-induced platelet clumping - Giant platelet - Platelet satellitism
36
Associated with decreased or ineffective megakaryopoiesis and thrombopoiesis.
Decreased Platelet Production
37
decreased platelet production marrow damage includes
o Aplastic Anemia o Fanconi’s Anemia (defect in DNA repair genes) o Malignancy (with or without myelophthisis)
38
decreased platelet production also deals with
- Congenital Defects (congenital thrombocytopenia) - Paroxysmal Nocturnal Hemoglobinuria (PNH)
39
decreased platelet production also deals with viral infections such as
o Rubella, CMV, EBV, HIV & Hep-C
40
decreased platelet production also deals with ineffective production such as
o Nutritional Deficiencies such as B12, Folate, Severe Fe deficiency.
41
decreased platelet production also deals with drugs such as
o Thiazides, Estrogen, Chemotherapy, Linezolid
42
decreased platelet production also deals with toxins such as
o Alcohol, Cocaine
43
decreased platelet production also deals with marrow failure (pancytopenia) such as
o Aplastic Anemia, Chemotherapy, Toxins
44
decreased platelet production also deals with B-12, Folate or _____ deficiency
Iron
45
decreased platelet production also deals with drugs that can selectively reduce platelet production such as
o Alcohol o Estrogens o Thiazides o Chlorpropamide o Interferon
46
decreased platelet production also deals with Amegakaryocytic Thrombocytopenia such as
o Myelodysplasia (pre-leukemia) o Immune (related to aplastic anemia)
47
decreased platelet production also deals with ______ and ________ thrombocytopenia
* Cystic thrombocytopenia (rare) * Inherited thrombocytopenia
48
TTP is a short term for
THROMBOTIC THROMBOCYTOPENIC PURPURA
49
TTP is characterized by __________ and ________________.
microangiopathic hemolytic anemia ; profound intravascular platelet clumping
50
Evaluation of suspected hereditary and acquired disorders of platelet function.
PLATELET AGGREGATION TESTING
51
Platelets normally contain three major types of granules
- alpha granules - dense granules - lambda granules
52
granule that contains fibrinogen, PF4, Factor V, Von Willebrand Factor.
Alpha Granules
53
granule that contains ADP or ATP, calcium and serotonin
Dense Granules
54
a granule that is similar to lysosomes and contain several hydrolytic enzymes.
o Lambda Granules
55
Hereditary platelet function disorders includes
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
Bernard Soulier Syndrome is a rare defects of _____
adhesion
57
Glanzmann Thrombasthenia is a rare defects of _____
aggregation
58
Alpha or Dense Granule Deficiency, Aspirin-like Defects, or Other primary defects is a more common defects of _______
secretion
59
Acquired platelet function disorders are more common than the _________ and include _________.
hereditary disorders ; drug-induced platelet dysfunction
60
drug-induced platelet dysfunction includes
o Aspirin o NSAID’s o Clopidogrel o Antibiotics o Various cardiovascular o Psychotropic Drugs o Uremia o Myeloproliferative disorders
61
TREATMENT OF PLATELET DISORDERS (Congenital Disorders)
- Platelet Transfusion = Leucodepleted, HLA compatible and Irradiated - DDAVP - Tranexamic Acid - Recombinant factor VIIa - Bone Marrow Transplantation

MT2 (45 decks)

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