Coagulation and Hematopathology Flashcards

1
Q

Primary hemostasis is accomplished by ______ and secondary hemostasis by ______

A
  • Platelets

- cascade fibrin formation

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2
Q

What forms platelets?

A

Megakaryocytes in bone marrow

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3
Q

What 4 things do platelets do to form a plug?

A

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

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4
Q

when thrombin is formed, soluble fibrinogen turns into what?

A

Insoluble fibrin clot

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5
Q

Which vitamin is crucial for coagulation?

A

Vitamin K

*facilitates creation of calcium binding sites

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6
Q

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

A

Fibrinolysis

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7
Q

What three basic tests are used to evaluate hemostasis?

A

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

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8
Q

PT measures ____ pathways and PTT measures ____ pathways

A

extrinsic (12-15 sec)

intrinsic (24-35 sec)

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9
Q

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

A

Thrombocytopenia

*decreased bone marrow production or sequestration in spleen

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10
Q

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

A

Inherited (von willebrand, hemophilia)

Acquired (liver disease, it K deficiency)

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11
Q

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

A

Von Willebrand factor (vWF)

*carrier for coagulation factor VIII

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12
Q

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

A

von Willebrand disease

*Type 1 (partial quantitative) most common

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13
Q

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

A

Hemophilia A

*prolonged PTT, normal PT and platelet

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14
Q

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

A

Hemophilia B

*prolonged PTT, normal PT and platelet

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15
Q

Massive systemic intravascular activation of coagulation is called?

A

Disseminated intravascular coagulation (DIC)

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16
Q

What is elevated in severe acute DIC?

A

d-dimer (fibrin degradation product)

*schistocytes, decreased platelets, hematocrit, fibrinogen

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17
Q

What is the pathologic counterpart of hemostasis?

A

Thrombosis

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18
Q

What are the 3 elements of Virchows triad?

A

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

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19
Q

What is the most common inherited thrombophilia in caucasians?

A

Factor V leiden

*single nucleotide change in coagulation factor V

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20
Q

How does factor V leiden affect clotting?

A

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

21
Q

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

A

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

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

22
Q

What are the two broad categories of WBC disorders?

A

1-Leukocytosis (too many)

2-Leukocytopenia (not enough)

23
Q

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

A

Lower than 1500 cells/ul

24
Q

Normal white blood cell counts are in what range?

A

3,500-10,000

25
Q

What are 4 causes of leukocytosis?

A

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

26
Q

What are the 3 size categories of anemia?

A

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

27
Q

What is the most common reason for microcytic anemia?

A

Iron deficiency

*usually because of bleeding

28
Q

What are 6 clinical features of iron deficiency anemia?

A
1-Pica (unusual craving)
2-Koilonychia (thin nails)
3-atrophic glossitis
4-esophageal web
5-pallor
6-weakness
29
Q

What are the two types of macrocytic anemia?

A

1-Non-megaloblastic

2-Megaloblastic

30
Q

What are the two likely causes of megaloblastic macrocytic anemia?

A

1-Folic acid deficiency (DNA synthesis)

2-B12 deficiency

31
Q

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

A

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

32
Q

What are 2 absorption requirements for Vitamin B12?

A

1-Intrinsic factor

2-Intact ileum

33
Q

Systemically distributed neoplasms of white cells are called?

A

Leukemia

34
Q

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

A

Lymphoma

35
Q

Lymphomas and leukemias are clonal expansions of cells at certain ________

A

Developmental stages

36
Q

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

A

Chronic (mutation in peripheral maturation)

Acute (mutation in bone marrow maturation)

37
Q

Leukemia is either ____ or ____ and _____ or _____

A

Acute or Chronic

Myeloid or Lymphoid

38
Q

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

A

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

39
Q

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

A

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

40
Q

Lymphomas are named based on what?

A

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

41
Q

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

A

Hodgkin Lymphoma

42
Q

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

A

Non-hodgkin lymphoma

*indolent/chronic generally not curable

43
Q

What are 3 clinical findings of lymphomas?

A

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

44
Q

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

A

1-Follicular lymphoma

2-Diffuse large B cell lymphoma

45
Q

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

A

Follicular Lymphoma

*bone marrow involvement common

46
Q

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

A

Diffuse Large B Cell Lymphoma

*peripheral blood involvement rare

47
Q

Which 4 factors are vitamin K dependent?

A

II, VII, IX, X

48
Q

Which factor is associated with von Willebrand disease?

A

VIII