Coagulation and Hematopathology

Question 1

Primary hemostasis is accomplished by ______ and secondary hemostasis by ______

Answer 1

• Platelets

- cascade fibrin formation

Question 2

What forms platelets?

Answer 2

Megakaryocytes in bone marrow

Question 3

What 4 things do platelets do to form a plug?

Answer 3

1-Adhere to exposed subendothelium/von willebrand factor
2-Secrete contents of granules
3-Aggregate with other platelets
4-Provide a procoagulant surface for coagulation cascade

Question 4

when thrombin is formed, soluble fibrinogen turns into what?

Answer 4

Insoluble fibrin clot

Question 5

Which vitamin is crucial for coagulation?

Answer 5

Vitamin K

*facilitates creation of calcium binding sites

Question 6

Mediated by plasmin, what is the break down of fibrin clot resulting in fibrin degradation products such as d-dimer called?

Answer 6

Fibrinolysis

Question 7

What three basic tests are used to evaluate hemostasis?

Answer 7

1-Platelets (count/morphology)
2-Coagulation (PT, PTT)
3-Fibrinolysis (d-dimer)

Question 8

PT measures ____ pathways and PTT measures ____ pathways

Answer 8

extrinsic (12-15 sec)

intrinsic (24-35 sec)

Question 9

A normal platelet count is between 150,000-450,000/uL, what is it called when it is decreased under 100,000?

Answer 9

Thrombocytopenia

*decreased bone marrow production or sequestration in spleen

Question 10

_____ abnormalities in coagulation factors affect a single factor, ____ usually affects multiple

Answer 10

Inherited (von willebrand, hemophilia)

Acquired (liver disease, it K deficiency)

Question 11

What protein mediates lately binding to sites of vascular injury by binding collagen and GP1b receptor?

Answer 11

Von Willebrand factor (vWF)

*carrier for coagulation factor VIII

Question 12

What condition is autosomal dominant, and has mucocutaneous platelet like bleeding and often has normal PTT?

Answer 12

von Willebrand disease

*Type 1 (partial quantitative) most common

Question 13

What condition is a factor VIII deficiency, has hematomas, hemorrhage in joints and easy bleeding?

Answer 13

Hemophilia A

*prolonged PTT, normal PT and platelet

Question 14

What condition is a factor IX deficiency, has hematomas, hemorrhage in joints and easy bleeding?

Answer 14

Hemophilia B

*prolonged PTT, normal PT and platelet

Question 15

Massive systemic intravascular activation of coagulation is called?

Answer 15

Disseminated intravascular coagulation (DIC)

Question 16

What is elevated in severe acute DIC?

Answer 16

d-dimer (fibrin degradation product)

*schistocytes, decreased platelets, hematocrit, fibrinogen

Question 17

What is the pathologic counterpart of hemostasis?

Answer 17

Thrombosis

Question 18

What are the 3 elements of Virchows triad?

Answer 18

1-Endothelial injury
2-Abnormal blood flow
3-Hypercoagulability of blood

Question 19

What is the most common inherited thrombophilia in caucasians?

Answer 19

Factor V leiden

*single nucleotide change in coagulation factor V

Question 20

How does factor V leiden affect clotting?

Answer 20

Protein C and protein S cannot degrade altered factor V, favoring fibrin formation and clotting

Question 21

What two things make up the majority of cells in a normal WBC?

Answer 21

1-Neutrophils (55%)
2-Lymphocytes (35%)

*eosinophils, basophils and monocytes are part of WBC as well

Question 22

What are the two broad categories of WBC disorders?

Answer 22

1-Leukocytosis (too many)

2-Leukocytopenia (not enough)

Question 23

At what level of neutropenia should the patient stay in the hospital?

Answer 23

Lower than 1500 cells/ul

Question 24

Normal white blood cell counts are in what range?

Answer 24

3,500-10,000

Question 25

What are 4 causes of leukocytosis?

Answer 25

1-Increased marrow production 2-Increased release of marrow stores 3-decreased margination 4-decreased extravasation into tissues

Question 26

What are the 3 size categories of anemia?

Answer 26

1-Microcytic Anemia (under 80) 2-Normocytic Anemia (80-100) 3-Macrocytic anemia (above 100)

Question 27

What is the most common reason for microcytic anemia?

Answer 27

Iron deficiency *usually because of bleeding

Question 28

What are 6 clinical features of iron deficiency anemia?

Answer 28

``` 1-Pica (unusual craving) 2-Koilonychia (thin nails) 3-atrophic glossitis 4-esophageal web 5-pallor 6-weakness ```

Question 29

What are the two types of macrocytic anemia?

Answer 29

1-Non-megaloblastic | 2-Megaloblastic

Question 30

What are the two likely causes of megaloblastic macrocytic anemia?

Answer 30

1-Folic acid deficiency (DNA synthesis) | 2-B12 deficiency

Question 31

What three things could cause folic acid deficiency and result in megaloblastic macrocytic anemia?

Answer 31

1-Poor diet 2-Decreased absorption (jejunal resection) 3-Increased utilization

Question 32

What are 2 absorption requirements for Vitamin B12?

Answer 32

1-Intrinsic factor | 2-Intact ileum

Question 33

Systemically distributed neoplasms of white cells are called?

Answer 33

Leukemia

Question 34

Solid tumors of hematopoietic system/neoplasms of lymphoid origin are called?

Answer 34

Lymphoma

Question 35

Lymphomas and leukemias are clonal expansions of cells at certain ________

Answer 35

Developmental stages

Question 36

If less than 20 % blasts in the periphery it is _____ vs more than it is _____

Answer 36

Chronic (mutation in peripheral maturation) Acute (mutation in bone marrow maturation)

Question 37

Leukemia is either ____ or ____ and _____ or _____

Answer 37

Acute or Chronic Myeloid or Lymphoid

Question 38

What 4 things go into go into making a diagnosis of leukemia?

Answer 38

1-Clinical Data 2-Phenotype 3-Genetic/molecular 4-Morphology

Question 39

What are the 3 sub groups of AML that the WHO recognizes?

Answer 39

1-Recurrent genetic abnormalities 2-Multilineage dysplasi 3-Not otherwise categorized

Question 40

Lymphomas are named based on what?

Answer 40

Where B cell development goes wrong. Whether in bone marrow or lymphoid tissue

Question 41

Which type of lymphoma is the minority, curable in most and has variable month-years survival?

Answer 41

Hodgkin Lymphoma

Question 42

Which type of lymphoma has chronic (years), aggressive (months) and very aggressive (weeks) types and is curable in some cases?

Answer 42

Non-hodgkin lymphoma *indolent/chronic generally not curable

Question 43

What are 3 clinical findings of lymphomas?

Answer 43

1-Enlarged, painless lymphadenopathy 2-B-symptoms (fever, weight loss) 3-Impingement or obstruction of other structures

Question 44

What are the two most common types of Non hodgkin lymphoma?

Answer 44

1-Follicular lymphoma | 2-Diffuse large B cell lymphoma

Question 45

Which lymphoma is the most common nonhodgkins lymphoma, occurs in at older age, is indolent and involves chromosomal translocation (14:18)?

Answer 45

Follicular Lymphoma *bone marrow involvement common

Question 46

Which type of nonhodgkins lymphoma is derived from B-cells, and is more likely to have extra nodal sites?

Answer 46

Diffuse Large B Cell Lymphoma *peripheral blood involvement rare

Question 47

Which 4 factors are vitamin K dependent?

Answer 47

II, VII, IX, X

Question 48

Which factor is associated with von Willebrand disease?

Answer 48

VIII