1Alternative name for Hereditary Hemorrhagic Telangiectasia is
Rendu-Osler-Weber Syndrome
2Classification of Hereditary Hemorrhagic Telangiectasia among vascular bleeding conditions is
Most common inherited vascular bleeding disorder
3Structural abnormality characteristic of Hereditary Hemorrhagic Telangiectasia involves
Dilatation of capillary walls
4Alternative name for Ehlers-Danlos Syndrome is
Cutis Hyperelastica
5Clinical manifestations associated with Ehlers-Danlos Syndrome include
Hyperextensible skin, hypermobile joints, joint laxity, fragile tissues, subcutaneous hematoma formation
6Alternative names for Henoch-Schonlein Purpura are
Allergic Purpura or Nonthrombocytopenic Purpura
7Patient population in which Henoch-Schonlein Purpura is most commonly seen is
Children
8Primary etiology of vascular abnormalities in Henoch-Schonlein Purpura is
Immunologic damage to endothelial cells
9Key symptoms characterizing Henoch-Schonlein Purpura include
Gastrointestinal hemorrhage and joint swelling
10Alternative name for Scurvy is
Ascorbic acid (Vitamin C) deficiency
11Defect characterizing Scurvy involves
Defects in the synthesis of collagen and hyaluronic acid
12Definition of thrombocytopenia is
Decreased platelet count
13Examples of conditions with impaired or decreased platelet production include
Bernard-Soulier syndrome, Fanconi anemia, TAR syndrome, viral infections, leukemia, megaloblastic anemias, WAS (Wiskott-Aldrich syndrome), MYH9 gene mutations
14Full name for TAR syndrome is
Thrombocytopenia with Absent Radius syndrome
15Example of MYH9 gene mutation condition is
May-Hegglin Anomaly
16Examples of conditions with increased platelet destruction include
HUS, DIC, ITP, TTP
17Full name for HUS is
Hemolytic uremic syndrome
18Full name for DIC is
Disseminated intravascular coagulation
19Full name for ITP is
Immune thrombocytopenic purpura
20Full name for TTP is
Thrombotic thrombocytopenic purpura
21Full name for HIT is
Heparin-induced thrombocytopenia
22Examples of conditions associated with increased splenic sequestration include
Gaucher disease, Hodgkin's disease, Sarcoidosis, Lymphoma, Cirrhosis of the liver, Portal hypertension
23Definition of thrombocytosis is
Increased platelet count
24Alternative name for Reactive Thrombocytosis is
Secondary thrombocytosis
25Core characteristic of Reactive Thrombocytosis is
Moderate increase in platelet count
26Examples of associated cases in Reactive Thrombocytosis include
Recovery from splenectomy, acute blood loss, major surgery
27Alternative name for Autonomous Thrombocytosis is
Primary thrombocytosis
28Core characteristic of Autonomous Thrombocytosis is
Marked increase in platelet count
29Examples of associated cases under Autonomous Thrombocytosis / MPNs include
Essential thrombocythemia, CML, Polycythemia vera, Primary myelofibrosis
30General definition of Myeloproliferative Neoplasms (MPNs) is
Group of malignant neoplasms described as clonal proliferations of bone marrow stem cells characterized by elevations in one or more myeloid cell types in peripheral blood
31Key characteristics of Essential Thrombocythemia include
Uncontrolled proliferation of bone marrow megakaryocytes with platelet count exceeding 1 million/µL
32Deficiency responsible for Bernard-Soulier Syndrome (BSS) is
GP Ib/IX/V
33Inheritance pattern of Bernard-Soulier Syndrome is
Autosomal recessive (AR)
34Features associated with Bernard-Soulier Syndrome include
Decreased platelet count, giant platelets, normal aggregation in ADP, abnormal aggregation in Ristocetin
35Key components important in platelet adhesion process are
GP Ib/IX/V (surface receptor) and VWF
36Definition of platelet adhesion is
Process wherein platelets adhere to a foreign surface
37Most common congenital bleeding disorder is
von Willebrand's Disease (vWD)
38General description of von Willebrand's Disease involves
Decreased Factor VIII, normal PT, prolonged APTT, abnormal aggregation with Ristocetin
39Bleeding time, bleeding tendency, and petechiae in Type I vWD are
Bleeding time normal or increased, mild bleeding tendency, no petechiae
40Bleeding time, bleeding tendency, and petechiae in Type II vWD are
Bleeding time increased, moderate bleeding tendency, usually no petechiae
41Bleeding time, bleeding tendency, and petechiae in Type III vWD are
Bleeding time increased, often severe bleeding tendency, occasionally petechiae
42Deficiency responsible for Glanzmann's thrombasthenia is
GP IIb/IIIa
43Features associated with Glanzmann's thrombasthenia include
Very prolonged bleeding time, abnormal clot retraction, abnormal aggregation response in ADP, normal aggregation response in Ristocetin
44Inheritance condition characterized by absence of fibrinogen is
Hereditary afibrinogenemia
45Definition of platelet aggregation is
Process wherein platelets adhere to other platelets
46Key components important in platelet aggregation process are
GP IIb/IIIa and Fibrinogen
47Examples of platelet secretion disorders include
Thromboxane pathway disorders and Storage pool diseases
48Deficiencies categorized under thromboxane pathway disorders are
Cyclooxygenase or thromboxane synthetase deficiency
49Two categories of storage pool diseases are
Alpha and Dense Granule Deficiencies
50Inheritance pattern of Gray Platelet Syndrome is
Autosomal recessive (AR)
51Specific structural absence in Gray Platelet Syndrome is
Absence of morphologically recognizable α-granules in thrombocytes
52Clinical and laboratory characteristics of Gray Platelet Syndrome include
Lifelong mild bleeding tendencies, moderate thrombocytopenia, fibrosis of the marrow, large gray platelets, increased plasma levels of PF-4 and β-thromboglobulin
53Inheritance pattern of Quebec platelet disorder is
Autosomal dominant (AD)
54Cause of Quebec platelet disorder is
Deficiency of multimerin (a protein stored in α-granules)
55Albinism presentation and platelet count in Hermansky-Pudlak syndrome are
Oculocutaneous albinism and normal platelet count
56Albinism presentation and platelet count in Chediak-Higashi syndrome are
Partial albinism and low platelet count
57Albinism presentation and platelet count in Wiskott-Aldrich syndrome are
No albinism and low platelet count
58Albinism presentation and platelet count in TAR syndrome are
No albinism and normal platelet count
59Inheritance pattern rule for dense granule deficiencies is
Autosomal Recessive (AR), except for Wiskott-Aldrich syndrome
60Most common acquired bleeding disorder is
Trauma-induced coagulopathy
61Inheritance pattern of Glanzmann's thrombasthenia is
Rare autosomal recessive disorder
62Comparative frequency of Glanzmann's thrombasthenia to BSS is
Occurs with lesser frequency than BSS
63Clot retraction in Glanzmann's thrombasthenia demonstrates
Abnormal clot retraction
64Platelet count and morphology in Glanzmann's thrombasthenia are
Normal platelet count and normal platelet morphology
65Expected bleeding time result in a patient with Glanzmann's thrombasthenia is
Prolonged bleeding time result