Sweep 1.1

Question 1

• Prothrombin time (PT)- measurement of time needed for plasma to form a clot in the presence of added

Answer 1

tissue thromboplastin and calcium ions

Question 2

o Prolonged PT- due to decrease/abnormalities in

Answer 2

Factors II, V, VII, X and/or fibrinogen

Question 3

PT: Measure degree of anticoagulation of patients on

Answer 3

Coumadin/Warfarin (I’m dumb, these are blood thinners)

Question 4

• Partial thromboplastin time (PTT)- time needed for plasma to form a clot in the presence of added

Answer 4

ground glass or kaolin, cephalin and calcium ions

Question 5

o Prolonged PTT- due to decrease/abnormalities of

Answer 5

Factors II, V, VIII, IX, X, XI, XII and/or fibrinogen

Question 6

PTT is a Measure of

Answer 6

heparin therapy

Question 7

Disorders of primary hemostasis-

o Clinical manifestations-

Answer 7

mucocutaneous bleeding, trauma associated bleeding

Question 8

Primary hemostasis disorders: o Lab manifestations-

Answer 8

prolonged bleeding time and thrombocytopenia

Question 9

• Disorders of secondary hemostasis-

o Clinical manifestations-

Answer 9

soft tissue bleeding, trauma associated bleeding

Question 10

Disorders of secondary hemostasis: o Lab manifestations-

Answer 10

prolonged PT and/or PTT and/or thrombin time

Question 11

• Disorders of regulatory system-

o Clinical manifestations-

Answer 11

soft tissue bleeding, trauma associated bleeding

Question 12

Disorders of regulatory system: o Lab manifestations-

Answer 12

normal

Question 13

• Factor VIII Complex- composed of ——–, associate

Answer 13

vWF, smaller multimers and factor VIII coagulant.

Question 14

Factor VIII complex: vWF functions both as a carrier molecule for —— and as glue between damaged ——-.

Answer 14

factor VIII, endothelium and platelets

Question 15

Factor VIII procoagulant is released from its association with

Answer 15

vWF when a clot is formed.

Question 16

Factor VIII: Participates in the generation of

Answer 16

Factor X. VIII association with vWF

Question 17

• von Willebrand Disease- ——– associated with ——–. Most common inherited bleeding disorder.

Answer 17

AD disorder, decreased production of vWF and by association decreased Factor VIII

Question 18

vWD: o Clinically-

Answer 18

mucocutaneous bleeding, nosebleeds, bruises, excessive menstrual blood flow; symptoms often improve after adolescence

Question 19

vWD: o Lab-

Answer 19

prolonged bleeding time, prolonged PTT, decreased vWF and decreased factor VIII

Question 20

vWD: o Therapy-

Answer 20

desmopressin, antifibrinolytic agents; factor VIII & vWF cryoprecipitate in severe cases

Question 21

• Hemophilia A- ———. due to decreased ——.

Answer 21

x-linked recessive, Factor VIII

Question 22

Hemophilila A: o Lab-

Answer 22

normal bleeding time, prolonged PTT, decreased VIII, normal vWF, normal IX. Severe cases <1% VIII, moderate 1-5%, mild >5%

Question 23

Hemophilila A: o Therapy-

Answer 23

replacement of factor VIII, desmopressin, fibrinolytic inhibitors

Question 24

• Hemophilia B- X-linked recessive due to decreases

Answer 24

Factor IX.

Question 25

Hemophilia B; Similar to hemophilia

Answer 25

A/factor VIII deficiency

Question 26

Hemophilia B: o Lab-

Answer 26

normal bleeding time, prolonged PTT, normal VIII and vWF and decreased factor IX

Question 27

• Immune Thrombocytopenic Purpura (ITP)-

Answer 27

GPIb/IX and IIb/IIIa are common targets.

Question 28

Immune thrombocytopenic purpura: Acute form occurs in

Answer 28

childhood, following viral prodrome, resulting in severe thrombocytopenia.

Question 29

Immune thrombocytopenic purpura: o Clinically-

Answer 29

petechial hemorrhage, bruising, gingival bleeding

Question 30

Immune thrombocytopenic purpura: o Lab-

Answer 30

bone marrow reveals megakaryocytes normal or increased

Question 31

Immune thrombocytopenic purpura: o Therapy-

Answer 31

acute often self-limited 2-6 weeks, CHRONIC: corticosteroids (suppress antibody formation), IV immunoglobulin, and splenectomy

Question 32

• Thombotic Thrombocytopenic Purpura (TTP)- acute disorder is characterized by

Answer 32

intravascular platelet activation with formation of platelet-rich microthrombi throughout circulation.

Question 33

TTP: Due to deficiency of metalloproteinase,

Answer 33

ADAMTS 13 that normally degrades very-high-molecular-weight multimers of von Willebrand factor.

Question 34

TTP: Results in accumulation of the abnormally large

Answer 34

vWF in the plasma.

Question 35

TTP: o Clinically-

Answer 35

fever, marked thrombocytopenia, microangiopathic hemolytic anemia, acute renal failure and neurologic changes

Question 36

TTP: o Lab-

Answer 36

thrombocytopenia, schistocytes in peripheral blood smear, anemia, reticulocytosis, increased bilirubin and LD.

Question 37

• Intrinsic defect hemolytic anemias-

Answer 37

hemoglobin production, membrane abnormality, inherited

Question 38

o Membrane defects (Hereditary spherocytosis)-

Answer 38

extravascular hemolysis. Inherited defect in the red cell membrane results in less deformability of RBC so that they are sequestered and destroyed in spleen.

Question 39

Hereditary spherocytosis: • Due to qualitative/quantitative deficiency of

Answer 39

spectrin (structural protein)

Question 40

• Wilm’s Tumor-

o Clinically-

Answer 40

abdominal mass, risk generally increased in some inherited syndromes, occurs in kids 2-5 yrs

Question 41

Wilm’s tumor:

Answer 41

o Path- triphasic pattern with epithelial structures resembling primitive tubules or glomeruli, stroma and blastemal which recapitulates early nephron formation; tumor illustrates abortive formation of renal structures in various stages of renal development

Question 42

• Renal Cell Carcinoma-

o Clinically-

Answer 42

2:1 male-female ratio; hematuria, mass, pain, fever, polycythemia, paraneoplastic syndrome

Question 43

Renal cell carcinoma: o Etiology-

Answer 43

risk factos include smoking, hypertension, obestity, cadmium exposure and von Hippel-Lindau syndrome

Question 44

Renal cell carcinoma: o Path-

Answer 44

arise from tubular epithelium, invasion of renal vein; clear cell carcinoma

Question 45

Renal cell carcinoma: o Therapy-

Answer 45

surgery +/- radiation

Question 46

Renal cell carcinoma: o Prognosis-

Answer 46

stage dependent 5 yr survival; stage 1- 81%; stage 4- 8%

Question 47

o Abnormal hemoglobin (sickle cell anemia)-

Answer 47

extravascular hemolysis. An inherited defect in structure of globin chain→ Valine for glutamic acid @ position 6 in beta chain to produce sickle hemoglobin

Question 48

Abnormal hemoglobin: • Can lead to

Answer 48

splenic sequestration, lead to infarct, ischemia

Question 49

Abnormal hemoglobin: • Clinically- occurs in homozygotes for

Answer 49

HbS; severe anemia, vaso-occlusive crises, acute chest syndrome and stroke

Question 50

(Thalassemia)- ——— that results in

Answer 50

Autosomal codominant, diminished or absent synthesis of either alpha or beta globin chains of hemoglobin.

Question 51

Thalassemia: Due to whole or partial

Answer 51

gene deletion as well as mutation or mRNA instability

Question 52

Thalassemia: • Clinically-

Answer 52

anemia, iron overload, severe transfusion-dependent anemia

Question 53

Thalassemia: • Precipitation or relative excess of other globin chain causes

Answer 53

membrane destruction and premature destruction of RBC precursors in marrow and spleen

Question 54

o G6PD def:

Answer 54

extravascular hemolysis: x-linked, primarily African Americans. Red cells are susceptible to oxidant injury. These are bite cells. Individuals are asymptomatic