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Hematology 2 > (F) Lesson 9: Vascular and Platelet Disorders > Flashcards

(F) Lesson 9: Vascular and Platelet Disorders Flashcards

1
Q

Vascular Disorders

Vascular disorders can be classified into either primary or secondary:

  1. There is a disease association
  2. There is no known disease associated
A
  1. Secondary
  2. Primary
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2
Q

Hereditary Vascular Disorders

  • Manifests with thin-walled vessels due to a lack of supporting tissues surrounding the vessels (e.g. smooth muscle deficiency, discontinued endothelial lining, etc.)
  • There is dilation of the vessels commonly seen in the lips and eyes
  • There is also the breakage of vessels in the nostrils
A

Hereditary Hemorrhagic Telangiectasia

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3
Q

Hereditary Vascular Disorders

Identify the Medical Terms:
1. Thin-walled vessels
2. Nosebleed

A
  1. Telangiectasia
  2. Epistaxis
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4
Q

Hereditary Vascular Disorders

What is the other name for Hereditary Hemorrhagic Telangiectasia?

A

Rendu-Osler-Weber Syndrome

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5
Q

Hereditary Vascular Disorders

When one is experiencing epistaxis (nosebleeding), there is a possible problem with hemostasis if:
1. The bleed requires ____ before it can be stopped
2. The duration is more than ____ minutes

A
  1. Mechanical Pressure
  2. 10 minutes
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6
Q

Hereditary Vascular Disorders

TOF: Epistaxis (nosebleeding) can only be seen in issues with the vessels

A

False (it also manifests with platelet problems)

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7
Q

Hereditary Vascular Disorders

If there is a deficiency with this control protein, the complement activity cannot be fully controlled leading to increased release of anaphylatoxins

Increased anaphylatoxins induce increased vascular permeability because these initiate an inflammatory response leading to bleeding

A

C1 Inhibitor

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8
Q

Hereditary Vascular Disorders

  • This is a form of bleeding diathesis/disorder
  • A vascular tumor presents as a tuft of capillaries which causes pooling of the platelets
  • Since it attracts platelets, thrombocytopenia occurs
A

Hemangioma-Thrombocytopenia Syndrome

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9
Q

Hereditary Vascular Disorders

What is the other name for Hemangioma-Thrombocytopenia Syndrome?

A

Kasabach-Merritt Syndrome

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10
Q

Hereditary Vascular Disorders

  • Can be an autosomal dominant/recessive/X-linked trait
  • Manifests with hyper-extensible skin, hyper-mobile joints, joint laxity, fragile tissue, and bleeding tendencies
A

Ehlers-Danlos Syndrome

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11
Q

Hereditary Vascular Disorders

  • The problem lies with the abnormal formation of collagen leading to the inability to support the skin and blood vessels
  • A lack of collagen decreases the number of available surfaces the platelets can bind to
  • Patients have a tendency to bruise easily as well as experience prolonged bleeding
A

Ehlers-Danlos Syndrome

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12
Q

Hereditary Vascular Disorders

  • Patients present with excessively long extremities and digits (not proportional to the torso)
  • There is a problem with the connective tissue (e.g. collagen, elastic fibers, and other supporting membranes)
  • Weakened blood vessels can balloon and lead to an aneurysm especially in the aorta
A

Marfan Syndrome

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13
Q

Hereditary Vascular Disorders

A connective tissue problem which manifests with weakened and soft bones

A

Osteogenic Imperfecta

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14
Q

Hereditary Vascular Disorders

  • There is a deficiency/defect with elastic fiber formation in the patient
  • It presents with striations in the skin and eyes
A

Pseudoxanthoma Elasticum

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15
Q

Acquired Vascular Disorders

  • This presents with a skin rash and edema due to drugs, food, insect bites, and vaccinations
  • Manifests as arthralgia, nephritis, abdominal pain, and purpuric skin lesions
  • The body produces IgA immune complexes which stick to blood vessels to induce inflammation leading to its destruction
A

Henoch-Schonlein Purpura

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16
Q

Acquired Vascular Disorders

What is the other name for Henoch-Schonlein Purpura?

A

Anaphylactoid/Allergic Purpura

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17
Q

Acquired Vascular Disorders

  • There is a coating of the platelet membranes leading to platelet adhesion and clot formation on the platelet surface
  • Certain proteins can be deposited on the the vessel walls which induce inflammatory responses (recruitment of phagocytes) that damage the vessels
A

Paraproteinemia and Amyloidosis

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18
Q

Acquired Vascular Disorders

Familiarize yourself with the proteins and conditions involved in Paraproteinemia and Amyloidosis

A

Myeloma proteins
- IgA and IgG myeloma
- Waldenstrom’s macroglobulinemia

Amyloid fibrous proteins
- Aggregated fibrils
- Purpura, hemorrhage, and thrombosis
- Abnormal platelet function

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19
Q

Acquired Vascular Disorders

  • Common in elderly men than women
  • There is a lack of collagen support for small vessels (increased capillary fragility) and a loss of subcutaneous fats and elastic fibers
  • Manifests with 1-10mm diameter dark blotches that do not blanch upon applying pressure
A

Senile Purpura

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20
Q

Acquired Vascular Disorders

In Senile Pupura, what are the expected lab results of the patients’ sample?

A

Normal with no other chemical bleeding manifestations

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21
Q

Acquired Vascular Disorders

  • There is decreased synthesis of collagen leading to weak capillary walls which presents with purpuric lesions
  • Manifests with bleeding gums, gingivitis, loss of teeth, pinpoint (petechial) hemorrhages on the hair follicles, fatigue, depression, increased susceptibility to infections, muscle weakness, joint bleeding, body aches, and many more
A

Scurvy (Vitamin C Deficiency)

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22
Q

Acquired Vascular Disorders

Vitamin C is one of the vitamins that we (can/cannot) naturally synthesize in our bodies

A

Cannot (hence, it is an essential vitamin)

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23
Q

Acquired Vascular Disorders

Refers to pinpoint hemorrhages on the hair follicle

A

Corkscrew Bleeding

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24
Q

Acquired Vascular Disorders

  • There is medication-induced vasculitis but still with functionally adequate platelets
  • Antibodies may develop against the vessel wall after taking the medication (formation of immune complexes) leading to increased vascular permeability
A

Drug-induced Vascular Purpura

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25
Q

Acquired Vascular Disorders

Familiarize yourself with the drugs involved in Drug-induced Vascular Purpura

A
  1. Aspirin
  2. Warfarin
  3. Barbiturates
  4. Diuretics
  5. Digoxin
  6. Methyldopa
  7. Antibiotics
  8. Sulfonamides
  9. Iodides
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26
Q

Vascular Disorders

According to the list, what are the 2 purpuras of unknown origin?

A
  1. Purpura Simplex
  2. Psychogenic Purpura
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27
Q

Platelet Disorders (Quantitative: Thrombocytosis)

  • Refers to the normal response of the body to produce more platelets due to blood loss and surgery (e.g. splenectomy)
  • IDA, inflammation and their diseases, stress, and exercise are common stimulants of this disorder
A

Reactive Thrombocytosis

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28
Q

Platelet Disorders (Quantitative: Thrombocytosis)

In Reactive Thrombocytosis:
1. TOF: When IDA normalizes, platelet count also returns back to normal
2. TOF: When we release APRs during inflammation, it tends to decrease platelets because it may reflect as an APR as well

A
  1. True
  2. False (increase)
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29
Q

Platelet Disorders (Quantitative: Thrombocytosis)

During blood loss, platelets will naturally decrease but due to this stimulant in the liver and bone marrow to release more, the platelet count will suddenly increase. What is this phenomenon called?

A

Rebound Thrombocytosis

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30
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

  • The uncontrolled production of blood cells because of a genetic abnormality in an enzyme that stimulates the BM to produce cells
  • The hyper-viscosity of the blood due to the increased RBC count can clog the vessels leading to platelet activation, thrombosis, and bleeding
A

Polycythemia Vera

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31
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

In Polycythemia Vera, what enzyme carries a genetic abnormality?

A

Tyrosine Kinase

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32
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

This type of cancer is due to the overactivity of BM cells

A

Chronic Myelogenous Leukemia (CML)

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33
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

  • Platelets and megakaryocytes are stimulated to release PDGF (alpha granules) which induces fibrous tissue formation (scar tissue replaces normal BM cells)
  • Commonly associated with tear-drop cells in the PBS and can also be a form of cancer
A

Myelofibrosis with Myeloid Metaplasia

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34
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

  • A condition wherein the platelet count reaches over 1,000 x 10^9/L (max. of 400-450)
  • It may lead to thrombosis (increased TXA2) and hemorrhage (lack of EPI response and decreased synthesis of ADP within the platelet)
A

Essential or Primary Thrombocythemia

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35
Q

Platelet Disorders (Quantitative: Myeloproliferative Disorders)

  1. (Increased/Decreased) TXA2 activates the platelet aggregation process
  2. Epinephrine and ADP are platelet aggregators, so a/an (increase/decrease) in these impairs the aggregation process
A
  1. Increased
  2. Decrease
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36
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

  • A genetic disorder where WBCs contain Dohle-like bodies accompanied by giant platelets leading to a decreased response to activating/aggregating agents
  • There is a mutation in the MYH9 gene which encodes non-muscle myosin heavy chains
A

May-Hegglin Anomaly

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37
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

  • There is a decrease in dense granule content resulting in decreased function
  • Megakaryocytes with abnormal ultrastructures increase in number
A

May-Hegglin Anomaly

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38
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

What gene is mutated in a May-Hegglin Anomaly?

A

MYH9 gene

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39
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

Is it a May-Hegglin Anomaly or Not?
Dohle bodies are seen accompanied by normal-looking platelets caused by trauma, injury, or infection

A

Not a May-Hegglin Anomaly

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40
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

These are blue spindles shown on the PBS after staining the slide with Wright’s stain

A

Pseudo-Dohle Bodies

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41
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

  • A genetic mutation or deletion of 200kbs in the RBM8A gene (1q21.1)
  • Presents with bony abnormalities, cardiac lesions, and leukemoid reactions (high immature WBC count)
A

Thrombocytopenia-Absent Radius (TAR) Syndrome

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42
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

  1. What gene is mutated or deleted in TAR syndrome?
  2. What area of the chromosome can it be found on?
  3. How many kbps are deleted?
A
  1. RBM8A
  2. 1q21.1
  3. 200kbps
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43
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Congenital

  • There is hypoplasia of the radial bones of the forearms with absent, short, or malformed ulnae
  • Platelet counts decrease by 10,000-30,000/µL in infancy
A

Thrombocytopenia-Absent Radius (TAR) Syndrome

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44
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Neonatal

  • Presents with a platelet count of < 150,000/µL depending on the period of life
  • Is present 1-5% of the time at birth; 75% of cases are already present on or within 72 hours of life
A

Neonatal Thrombocytopenia

45
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Neonatal

Familiarize yourself with the causes of Neonatal Thrombocytopenia once it is already present during the fetal stage

A
  1. Congenital infections (TORCH and HIV)
  2. Trisomy and triploidy (aneuploidy)
  3. May be alloimmune or autoimmune
  4. HDN, Wiskott-Aldrich Syndrome, and drugs (chlorothiazide diuretics and tolbutamide)
46
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Impaired or Decreased Production: Acquired

  1. ____ prevents folic acid absorption which is important for DNA synthesis leading to decreased platelet count
  2. If you have ____ anemia due to lack of folate, there is an observed decrease in blood cell count
A
  1. Ethanol
  2. Megaloblastic anemia
47
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

  • Refers to when antibodies produced due to viral infections/live attenuated vaccines also cross react with the platelets leading to their destruction
  • Can be acute or chronic
A

Immune Thrombocytopenic Purpura (ITP)

48
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune (Acute or Chronic)

  • Presents with bruising, petechiae, and mucosal bleeding (epistaxis)
  • Is commonly seen in children and may be self-limiting but remissions are possible in 80% of the cases
49
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune (Acute or Chronic)

  • Presents with mucocutaneous bleeding, menorrhagia for females, recurrent epistaxis, and easy bruising
  • Is commonly seen in later ages such as 20 to 40 years old
50
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

Familiarize yourself with the platelet components targetted by the autoantibodies produced in Immune Thrombocytopenic Purpura (ITP)

A
  • GP IIB and IIIA
  • GP IA and IIA
  • IgG antibodies
51
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

Familiarize yourself with the 4 mechanisms of Drug-Induced Thrombocytopenia

A
  1. Hapten-induced
  2. Drug-dependent
  3. Drug-induced autoantibodies
  4. Immune complexes
52
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Drug-Induced Thrombocytopenia Mechanism

After intaking the drugs, the body metabolizes it which mixes with your plasma proteins turning into a complete antigen which can trigger an immune response

A

Hapten-Induced

53
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Drug-Induced Thrombocytopenia Mechanism

  • An antigen-antibody complex gets formed and the Fab portion of the quinine-dependent antibody will bind to GPIX of the platelet which appears like a new protein capable of triggering an immune response
  • Since the Fc region of the antibody is exposed, macrophages are able to attach to it
A

Drug-dependent

54
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Drug-Induced Thrombocytopenia Mechanism

Antibodies are produced for both the drugs and the cells (e.g. gold salts, procainamide, etc.)

A

Drug-induced Autoantibodies

55
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Drug-Induced Thrombocytopenia Mechanism

An example of this is Heparin-Induced Thrombocytopenia (HIT)

A

Immune Complexes

56
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Heparin-Induced Thrombocytopenia (HIT)

What is its other name?

A

“Heparin-Induced Thrombotic Thrombocytopenic Syndrome”

57
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Heparin-Induced Thrombocytopenia (HIT)

  • Excessive amounts of heparin can lead to the binding of ____ which is responsible for neutralizing the heparin that is naturally present in our bodies to continue with clotting
  • This leads to the formation of new proteins/antigens which leads to the production of ____ antibodies
A
  1. Platelet Factor 4 (PF4)
  2. HIT antibodies
58
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Heparin-Induced Thrombocytopenia (HIT)

  • The ____ regions of the HIT antibodies will bind to the platelets and induce clumping (thrombus formation)
  • ____ occurs due to the platelets being used up for the thrombus formation
A
  1. Fc
  2. Thrombocytopenia
59
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Tests for Heparin-Induced Thrombocytopenia (HIT)

  • A platelet count presenting with a ____% decrease from the baseline may signal HIT, even if its within the reference interval
  • (Antigen/Antibody) tests such as ELISA, rapid test, and coagulation-based tests are performed to check for the presence of the HIT complex
A
  1. 30%
  2. Antigen
60
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Tests for Heparin-Induced Thrombocytopenia (HIT)

  • Refers to the mixing of washed platelets (PRP) with the radioactive variant of this chemical in order to be absorbed
  • Upon adding a plasma sample of a patient suspected to have HIT, if the complex is present, it will activate the platelet leading to the release of this
  • This is measured using a scintillation counter
A

Serotonin-Release Assay

61
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Tests for Heparin-Induced Thrombocytopenia (HIT)

What is the gold standard test for HIT?

A

Serotonin-Release Assay

62
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Tests for Heparin-Induced Thrombocytopenia (HIT)

PRP is mixed with a plasma sample from a patient suspected of having HIT and the (+) result is the presence of platelet aggregates

A

Heparin-induced Platelet Aggregation Test (HIPA)

63
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

  • Similar to HDN wherein the mother is (-) for the platelet antigen but the baby is (+)
  • If there is an interaction, the mother will produce antibodies (IgGs) against the baby’s antigens which will then cross the placenta and attack the baby’s cells
A

Neonatal Alloimmune Thrombocytopenia

64
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

  • This may appear at birth but manifests as hemorrhage later on in life
  • Patients can recover within a 1-2 week period
  • Corticosteroids (immunosuppressants) dampen the immune activity of the mother to prevent further production of antibodies
A

Neonatal Alloimmune Thrombocytopenia

65
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

  • The mother has an autoimmune condition such as ITP or SLE (e.g. antinuclear antibodies)
  • The IgGs may cross the placenta and affect the platelets
  • Platelet count may appear decreased/normal at birth and may progress for about several weeks (1 to 2 weeks or several months) before it increases
A

Neonatal Autoimmune Thrombocytopenia

66
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

  • The development of platelet antibodies after receiving platelets containing blood products
  • Is generally self-limiting and may recover after 21 days but 10-15% of patients have been reported to have died
A

Post-Transfusion Purpura (PTP)

67
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

In blood banking practice, it is better to have the platelet source come from who to prevent the production of antibodies?

A

1 person (single platelet donation through apheresis)

68
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

If antibodies against platelets are already present but the patient is in dire need of transfused platelets, perform this test

A

HLA compatibility (with ABO as the minimum compatibility requirement)

69
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

The phenomenon of despite receiving platelets through donation, the patient’s platelet count is still decreased due to antibodies

A

Platelet Refractoriness

70
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Immune

Familiarize yourself with the antibodies against platelet antigens

A
  1. P1A1 (HPA-1a)
  2. P1A2, Bak-a and Bak-b
  3. Pen-a
  4. Yuk
  5. Br-a
71
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Non-Immune

  • Presents with blood vessels producing many clots which impedes blood flow leading to damaged platelets due to turbulence
  • Schistocytes and fragmented RBCs (due to the squeezing) can also be seen in the PBS
A

Microangiopathic Syndromes

72
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Non-Immune

What is the hallmark for Microangiopathic Syndromes?

A

Schistocytes in the PBS

73
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Microangiopathic Syndromes

  • There is a lack of ADAMTS-13, a disintegrin and metalloprotease with a thrombospondin type 1 motif
  • It controls the activity of vWF by fragmenting and cutting it during times of platelet adhesion
A

Thrombotic Thrombocytopenic Purpura (TTP)

74
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Microangiopathic Syndromes

  • E. coli OH serotype: 0157-H7 binds or produces a Shiga-like toxin (SLT-1 or 2) that leads to damaged renal vessels
  • The bacteria can attach using their pili which damages the vessel walls leading to the formation of a clot alongside increased blood urea
A

Hemolytic Uremic Syndrome (HUS)

75
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Microangiopathic Syndromes

  • A form of consumptive coagulation (it consumes clotting factors, fibrinogen, and platelets)
  • There is activation of the clotting mechanism secondary to trauma, infection, disease, or post-pregnancy
A

Disseminated Intravascular Coagulation (DIC)

76
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Other Causes

These two (2) conditions can be due to lordosis

A
  1. Pre-eclampsia
  2. Gestational thrombocytopenia
77
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Increased Platelet Destruction: Other Causes

Refers to thrombus development confined within the liver (similar with DIC)

A

Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) Syndrome

78
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Abnormal Distrubution

  • 70% of platelets are circulating in the blood while 30% are sequestered in the spleen
  • If the spleen enlarges, more platelets will be sequestered/stored in the spleen leading to decreased platelet count in the blood
A

Increased Platelet Sequestration

79
Q

Platelet Disorders (Quantitative: Thrombocytopenia)

Abnormal Distrubution

It induces the pooling of platelets to the site of injury therefore decreasing the number of circulating platelets

A

Kasabach-Merritt Syndrome

80
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

A defect in GPIIB/IIIA which is important for normal platelet aggregation

A

Glanzmann-Thrombasthenia

81
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

  • A defect in the GP1B95 complex which is important for normal platelet adhesion
  • Can be considered both a quantitative and qualitative platelet disorder
A

Bernard-Soulier Syndrome

82
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

TOF: If a patient is unresponsive to Ristocetin, it can also be due to VWF disease, not just Bernard-Soulier Syndrome

83
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

Refers to the impairment in the cleavage of the contractile microtubules (cytoskeleton) for the release of platelet granules

A

Caplain Defect

84
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

This can be due to the ff. conditions:
- Autoantibodies against GP IIb/IIIa
- Paraproteins against GP IIIa making platelet aggregation impossible
- Afibrinogenemia

A

Thromabasthenia-like Conditions

85
Q

Platelet Disorders (Qualitative: Disorders of Surface Membranes)

In Platelet-type VWD, a patient tests positive or negative for Bernard-Soulier Syndrome?

A

Positive (+)

86
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Can manifest as problems with either the alpha or dense granules

A

Storage Pool Disease

87
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Storage Pool Disease: Alpha or Dense Granules?

Gray Platelet Syndrome

A

Alpha Granules

88
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Storage Pool Disease

Refers to the cytoplasmic color of platelets after staining it with Wright’s stain

A

Gray Platelet Syndrome

89
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Storage Pool Disease: Alpha or Dense Granules?

  • Leads to unresponsiveness to aggregating agents even with a normal platelet count
  • A response to the agents will only produce primary waves, not secondary waves which is still considered deficient
A

Dense Granules

90
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Familiarize yourself with the 4 conditions associated with dense granule problems in Storage Pool Disease

A
  1. Hermansky-Puldak syndrome
  2. Chediak-Higashi syndrome
  3. Wiscott-Aldrich syndrome
  4. Thrombocytopenia with absent radii (TAR) syndrome
91
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Storage Pool Disease

Swiss Cheese Platelet refers to the ____ of platelet microtubules

92
Q

Platelet Disorders (Qualitative: Disorders of Platelet Secretion)

Storage Pool Disease

  • There is a problem with the development of nitric oxide leading to the digestion of granules and eventual impairment of adhesion
  • The gene defective in this disorder is responsible for controlling the release of urea
A

Quebec Platelet Disorder

93
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

A defect in α2β1 (GP Ia/IIa) and GPVI results to poor platelet response for this vessel component

A

Collagen Receptors

94
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

  • Refers to P2X1, P2Y1, and P2Y12
  • A lack of G-inhibitory complex formation results in poor response to this substance but with a normal platelet shape change and calcium mobilization
A

ADP Receptors

95
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

  • This is part of the STD receptor
  • Its defect leads to poor response by the platelets
A

Epinephrine Receptors

96
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

  • Various intracellular signaling defects affect the G-protein sub-unit and the phospholipase C isoenzyme
  • This specific G-pathway stimulates thrombosthenin to make the platelets contract and release their content
A

Calcium Mobilization Defects

97
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

  • There is a lack of thrombin generation and calcium-induced PL scrambling
  • Platelet plug formation is normal but the clotting factors have poor assembly on the platelet surface
A

Scott Syndrome

98
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

This factor is important for platelet shape change to expose the phospholipid which is an important cofactor for Vitamin-K dependent clotting factors

99
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

There is a defect in amino phospholipase translocase hence platelets are always in an activated state

A

Stormorken Syndrome

100
Q

Platelet Disorders (Qualitative: Disorders of Receptors and Pathways)

This defective enzyme in Stormorken Syndrome is responsible for decreasing cAMP activity which increases ADP leading to platelet activation

A

Amino Phospholipase Translocase

101
Q

Platelet Disorders (Qualitative: Acquired Type)

Phosphodiesterase (PDE) is important for the normal formation of ____ and release of ____

A
  1. Formation of Prostaglandin
  2. Release of Thromboxane
102
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

Refers to Polycythemia Vera (PV), Chronic Myelogenous Leukemia (CML), Myelofibrosis with Myeloid Metaplasia (MMM), and Essential Thrombocythemia (ET)

A

Myeloproliferative Neoplasm

103
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

Refers to when platelets are coated with paraproteins

A

Multiple Myeloma and Waldenstrom Macroglobulinemia

104
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

In Uremia:
1. Excess urea in the blood releases ____ which is a nitric oxide donor
2. The nitric oxide increases ____ inhibiting platelet activation

A
  1. Guanidinosuccinic acid (GSA)
  2. Guanylate cyclase
105
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

In uremia, the enzymes are important for the reduction of cAMP activity but since the cAMP will increase in this case, ADP will (increase/decrease) which inhibits platelet function

106
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

This causes a defect/lack in platelet aggregation

A

Hereditary Afibrinogenemia

107
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

In Hyper-Aggregable Platelets:
There is a hyper-aggregation response to either ADP or epinephrine

A

Sticky Platelet Syndrome

108
Q

Platelet Disorders (Qualitative: Secondary to Other Diseases)

In Hyper-Aggregable Platelets:
There is aggregation during in-vitro stirring

A

Spontaneous Aggregation

Hematology 2 (10 decks)

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