Platelet Disorders

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1
Identify the three primary reasons why platelets are difficult to count manually.
1. Adhere to foreign surfaces, 2. Easily disintegrate, 3. Hard to differentiate from debris.
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2
True or False: Platelets are evenly distributed in the blood because they tend to clump.
False.
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3
Fill in the blank: The reference method for manual platelet counting described by Brecher and Cronkite is the ________ method.
Phase-contrast microscope.
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4
Identify the specific diluent used in the Phase-contrast microscope method.
-1% ammonium oxalate.
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5
List the three physical characteristics of a platelet as seen under a microscope.
-2 to 4 µm diameter, round or oval shape, and light purple sheen.
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6
Fill in the blank: During manual counting, "________" erythrocytes are frequently seen in the background.
Ghost.
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7
Identify the specific area of the hemocytometer where platelets are counted.
-25 small squares of the central large square (1 mm²).
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8
True or False: The difference between the total cells counted on each side of the counting chamber should be less than 10%.
True.
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9
Identify the requirement if the percentage difference between the two sides of a hemocytometer is greater than 10%.
Repeat the procedure.
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10
Provide the formula for calculating the percentage difference between two counting sides.
% diff = [(V1 - V2) / ((V1 + V2) / 2)] x 100.
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11
Write the general formula used for manual cell counts per µL.
(cells counted x dilution factor) / (area in mm² x depth 0.1).
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12
Identify the purpose of performing a platelet count estimation.
To countercheck the results of the machine.
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13
Fill in the blank: To estimate platelets, one must examine the thin area of a wedge blood smear using the ________ objective.
Oil immersion (100x).
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14
Identify the normal range of platelets seen per 100x field in a wedge blood smear.
-7 to 21 cells.
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15
List the steps to calculate the estimated platelet count per µL using the standard method.
-1. Scan 10 oil immersion fields, 2. Average the number of platelets per field, 3. Multiply by 20,000.
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16
Provide the formula for platelet estimation used in cases of significant anemia or erythrocytosis.
(Average no. of platelets per field x total RBC count) / 200 RBCs per field.
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17
Identify the platelet estimate report category for a count of 50,000 to 99,000/µL.
Moderate decrease.
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18
Identify the platelet estimate report category for a count exceeding 800,000/µL.
Marked increase.
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19
True or False: A platelet count of 100 x 10⁹/L is considered a slight decrease.
True.
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20
Identify the target platelet count range required for Platelet-Rich Plasma (PRP) in aggregometry.
-200,000 to 300,000/µL.
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21
List the centrifugation parameters used to prepare PRP from sodium citrate-anticoagulated blood.
-50 x g for 30 minutes.
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22
Identify the storage temperature and timeframe for PRP-based light-transmittance aggregometry.
-18 to 24°C; start no less than 30 minutes after centrifugation and complete within 4 hours of collection.
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23
True or False: Light-transmittance aggregometry is considered reliable even when the whole-blood platelet count is 100,000/µL.
False.
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24
Identify the aggregating reagent that results in an abnormal response for both Bernard Soulier Syndrome (BSS) and von Willebrand's Disease (VWD).
Ristocetin.
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25
Fill in the blank: Platelet lumi-aggregometry measures platelet aggregation and the release of ________ from dense granules.
ATP (adenosine triphosphate).
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26
Identify the reagent used in lumi-aggregometry that produces chemiluminescence when oxidized by ATP.
Firefly derived luciferin-luciferase.
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27
True or False: In vascular disorders, the platelet count and coagulation tests usually yield normal results.
True.
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28
Identify the two tests that typically yield abnormal results in vascular disorders.
Bleeding time and Rumple-Leede test.
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29
Identify the most common inherited vascular bleeding disorder characterized by localized dilation of capillary walls.
Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome).
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30
List three clinical characteristics of Ehlers-Danlos Syndrome.
Hyperextensible skin, hypermobile joints, and fragile tissues.
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31
Identify the vascular disorder most commonly seen in children, characterized by immunologic damage to endothelial cells and GI hemorrhage.
Henoch-Schonlein Purpura.
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32
Fill in the blank: Scurvy is caused by a deficiency in ________, which leads to defects in the synthesis of collagen and hyaluronic acid.
Vitamin C (ascorbic acid).
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33
List four conditions associated with impaired or decreased platelet production.
Fanconi anemia, TAR syndrome, Megaloblastic anemias, and Wiskott-Aldrich syndrome.
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34
Identify the specific gene mutation associated with May-Hegglin Anomaly.
MYH9.
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35
List four conditions associated with increased platelet destruction.
HUS, DIC, ITP, and TTP.
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36
Fill in the blank: In Heparin-induced thrombocytopenia (HIT), platelets are activated by IgG antibodies against a complex of heparin and ________.
PF-4.
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37
List four conditions that lead to increased splenic sequestration of platelets.
Gaucher disease, Hodgkin's disease, Sarcoidosis, and Cirrhosis of the liver.
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38
Identify the alternative name for Reactive Thrombocytosis.
Secondary Thrombocytosis.
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39
List the four Myeloproliferative Neoplasms (MPNs) associated with Essential (Primary) Thrombocytosis.
-1. Chronic Myelogenous Leukemia (CML), 2. Polycythemia Vera, 3. Primary Myelofibrosis, 4. Essential Thrombocythemia.
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40
Identify the platelet count threshold often exceeded in Essential Thrombocythemia.
-1 million/µL.
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41
Identify the deficiency associated with Bernard Soulier Syndrome.
GP Ib/IX/V.
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42
True or False: Bernard Soulier Syndrome is characterized by giant platelets and abnormal aggregation in Ristocetin.
True.
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43
Identify the most common congenital bleeding disorder.
von Willebrand's Disease (VWD).
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44
List the primary platelet surface receptor and the ligand involved in platelet adhesion.
GP Ib/IX/V (receptor) and VWF (ligand).
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45
Identify the inheritance pattern of Type 1 von Willebrand's Disease.
Autosomal Dominant.
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46
Identify the deficiency associated with Glanzmann's thrombasthenia.
GP IIb/IIIa.
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47
Identify the specific laboratory finding that distinguishes Glanzmann's thrombasthenia from BSS regarding clot retraction.
Abnormal clot retraction.
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48
Fill in the blank: Platelet aggregation is the process where platelets adhere to other platelets, requiring the ________ receptor and the ________ ligand.
GP IIb/IIIa; Fibrinogen.
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49
Identify the disorder characterized by the specific absence of alpha-granules, resulting in large, gray platelets.
Gray Platelet Syndrome.
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50
List three dense granule deficiencies associated with albinism.
Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, and Wiskott-Aldrich syndrome.
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51
True or False: The inheritance pattern for most dense granule deficiencies is Autosomal Recessive, except for Wiskott-Aldrich syndrome.
True.
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52
Identify the most common acquired bleeding disorder according to Rodak's Hematology.
Trauma-induced coagulopathy.
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53
True or False: Glanzmann's thrombasthenia typically demonstrates a normal platelet count and normal platelet morphology.
True.
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54
Fill in the blank: For vascular disorders, the expected laboratory results for platelet count, platelet function tests, and coagulation tests are typically ________.::
NORMAL
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55
Identify the two specific tests that yield abnormal results in primary hemostasis disorders of the blood vessels.::
BLEEDING TIME, RUMPLE-LEEDE TEST
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56
For Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome), identify the inheritance pattern and its primary vascular characteristic.::
AUTOSOMAL DOMINANT (AD); LOCALIZED DILATION OF CAPILLARY WALLS
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57
Identify the vascular disorder also known as Cutis Hyperelastica, which is characterized by hyperextensible skin and fragile tissues.::
Ehlers-Danlos Syndrome
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58
For Henoch-Schonlein Purpura, identify the most common patient demographic and the likely cause of vascular abnormalities.::
Children; immunologic damage to the endothelial cells
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59
Identify the clinical features of Henoch-Schonlein Purpura regarding the gastrointestinal tract and joints.::
Gastrointestinal hemorrhage and joint swelling
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60
Fill in the blank: Scurvy, or Vitamin C deficiency, is characterized by defects in the synthesis of ________ and ________.::
collagen; hyaluronic acid
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61
For quantitative platelet disorders, enumerate four conditions associated with impaired or decreased platelet production.::
Fanconi anemia, Inherited Aplastic Anemia, TAR syndrome, Megaloblastic anemias
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62
Identify the specific gene mutation associated with May-Hegglin Anomaly.::
MYH9
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63
Enumerate four examples of conditions characterized by increased platelet destruction.::
HUS, DIC, ITP, TTP
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64
For Heparin-induced thrombocytopenia (HIT), identify the type of antibodies that activate platelets and the complex they target.::
IgG; heparin and PF-4
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65
Enumerate five conditions or factors associated with increased splenic sequestration of platelets.::
Gaucher disease, Hodgkin's disease, Sarcoidosis, Lymphoma, Cirrhosis of the liver
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66
Identify the alternative name for Reactive Thrombocytosis and its characteristic platelet count level.::
SECONDARY THROMBOCYTOSIS; MODERATELY HIGH
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67
Enumerate the four examples of Myeloproliferative Neoplasms (MPNs) associated with primary thrombocytosis.::
Chronic Myelogenous Leukemia (CML), Polycythemia Vera, Primary Myelofibrosis, Essential Thrombocythemia
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68
Identify the platelet count threshold often reached in Essential Thrombocythemia.::
Exceeding 1-million/µl
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69
For Bernard Soulier Syndrome (BSS), identify the deficient glycoprotein and the inheritance pattern.::
GP Ib/IX/V; Autosomal Recessive (AR)
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70
Identify the three characteristic laboratory findings for Bernard Soulier Syndrome regarding platelet size and aggregation.::
Giant Platelet, Abnormal Aggregation in Ristocetin, Normal Aggregation in ADP/Collagen/Epinephrine
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71
Identify the most common congenital bleeding disorder.::
von Willebrand's Disease (VWD)
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72
For the process of platelet adhesion, identify the primary platelet surface receptor and the required ligand.::
GP Ib/IX/V (receptor); VWF (ligand)
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73
Identify the inheritance pattern of Type 1 VWD and its typical aggregation response to Ristocetin.::
Autosomal Dominant; Abnormal Aggregation
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74
For Glanzmann's thrombasthenia, identify the deficient glycoprotein and its inheritance pattern.::
GP IIb/IIIa; Autosomal Recessive
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75
Identify the three characteristic laboratory findings for Glanzmann's thrombasthenia regarding clot retraction and aggregation.::
Abnormal Clot Retraction, Normal Aggregation with Ristocetin, Abnormal Aggregation with other reagents
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76
For the process of platelet aggregation, identify the specific platelet fibrinogen receptor and the ligand.::
GP IIb/IIIa (receptor); Fibrinogen (ligand)
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77
Identify the specific storage pool disease characterized by the absence of alpha-granules and the presence of large, gray platelets.::
Gray Platelet Syndrome
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78
For Gray Platelet Syndrome, identify the expected plasma levels of PF-4 and beta-thromboglobulin.::
INCREASED
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79
Identify the autosomal dominant bleeding disorder resulting from a deficiency of multimerin.::
Quebec platelet disorder
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80
Enumerate the three dense granule deficiencies associated with some form of albinism.::
Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, Griscelli syndrome
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81
Identify the dense granule deficiency that is inherited in an X-linked manner rather than autosomal recessive.::
Wiskott-Aldrich syndrome
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82
Identify the most common acquired bleeding disorder according to Rodak's Hematology.::
Trauma-induced coagulopathy
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83
True or False: Glanzmann's thrombasthenia occurs with higher frequency than Bernard Soulier Syndrome.::
False
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84
True or False: A patient with Glanzmann's thrombasthenia is expected to have a normal platelet count and normal platelet morphology.::
True
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