Module 18- Blood Disorder part 2 Flashcards

1
Q

What is the most common genetic disorder of the blood?

A

Sickle Cell Disease

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2
Q

Sickle Cell Disease is a ________ form of hemolytic anemia?

A

hereditary

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3
Q

What ethnicities are primarily affected by sickle cell disease?

A

African American

Mediterranean

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4
Q

What test is done to see if you are a sickle cell carrier?

A

Electrophoresis

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5
Q

Signs and symptoms of sickle cell disease start appearing within the first ______mo with ____ and _____ observed?

A

6
anemia
vasculopathy

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6
Q

Sickle cell disease is due to ______ hemoglobin

A

defective

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7
Q

In sickle cell disease fluid ______ results and blood _____ occurs?

A

viscosity

stasis

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8
Q

Due to blood viscosity and blood stasis _____ and ______ can result?

A

thrombosis

infarction

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9
Q

A hematocrit score for a sicle cell pt. can range between ____ and ___%

A

18

30

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10
Q

Normal life span of RBC?

A

90-120 days

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11
Q

Life span of a sickle cell RBC?

A

10-15

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12
Q

Which form of sickle cell disease do children and adolescence have?

A

Sickle Cell Crisis

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13
Q

What do we as clinicians do if someone experiences a sickle cell crisis?

A

stop tx
call med services
administer oxygen

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14
Q

What is the major organ affected in sickle cell disease?

A

kidney

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15
Q

What procedure may provide a cure for young patients with symptomatic sickle cell disease?

A

allogeneic stem-cell transplant

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16
Q

What do sickle cell pts. take daily until age 6 to prevent infection

A

penicillin

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17
Q

You could use a chemotherapy med called ___ to increase ______ thereby decreasing the permanent formation of sickle cells?

A

hydroxyurea

hemoglobin F

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18
Q

There is a decreased ______ and an increased _____ seen radiographically with Sickle Cell Disease?

A

radiodensity

osteoporosis

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19
Q

Sickle Cell Disease can present radiographically as a _____ trabecular pattern appearing as “____ _____” rows with large _____ spaces?

A

coarse
step-ladder (horizontal)
marrow

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20
Q

Sickle Cell pts. will have a generalized ______ of the oral tissues?

A

pallor

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21
Q

______ ______ of the mouth can lead to a sickle cell crisis

A

periodontal involvement

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22
Q

Do we use epi in LA on sickle cell pts.?

A

low dose

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23
Q

Can we use nitrous on sickle cell pts?

A

yes with oxygen greater than 50%

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24
Q

What does polycythemia mean?

A

increase in number and concentration of RBC above normal

25
Q

Hemoglobin and Hematocrit are increased to more than ____% in polycythemias?

A

50-55%

26
Q

What are the 3 general categories of polycythemia?

A

relative
primary
secondary

27
Q

In relative polycythemia we loose ____ which leads to a corresponding loss of ____

A

plasma level

RBC’s

28
Q

What can cause relative polycythemia (fluid loss)

A

dehydration
diarrhea
vomiting
sweating

29
Q

In relative polycythemia the ______ of cells increases due to?

A

concentration

loss of plasma level

30
Q

Which polycythemia is an actual increase in RBC count and hemoglobin value?

A

Polycythemia Vera (primary polycythemia)

31
Q

What else is elevated in polycythemia vera?

A

WBC

platelets

32
Q

Blood viscosity ________ in polycythemia vera?

A

increases

33
Q

Polycythemia Vera is a _____ condition resulting from a _____ disorder in which _____ RBC’s proliferate?

A

neoplastic
bone
primitive (reticulocytes)

34
Q

The tongue, mucous membranes, and gingiva are what color in polycythemia vera?

A

purplish-red

35
Q

What is the treatment for polycythemia vera?

A

chemo
radiation
phlebotomy

36
Q

Pts should avoid ____ supplements and ____ medications with a history of polycythemia vera?

A

iron

aspirin

37
Q

Secondary polycythemia is also called ____

A

erythrocytosis

38
Q

What patients can have a secondary polycythemia?

A

COPD
smokers
heart conditions

39
Q

A decrease in the total number of WBC’s is termed a?

A

leukopenia

40
Q

What are the 2 conditions that can lead to leukopenia?

A

infections

Disease/intoxification of bone marrow

41
Q

What is a rare, serious disease involving the destruction of bone marrow

A

Malignant Neutropenia

42
Q

What is the term for a malignant neutropenia?

A

Agranulocytosis

43
Q

What is the most extreme abnormal cause of leukocytosis?

A

leukemia

44
Q

What is often administered to a patient with lymphocytopenia?

A

gamma globulins

45
Q

Bleeding disorders can be due to platelet _____ or ______

A
dysfunction
numbers (low)
46
Q

Where are the clotting factors produced?

A

liver

47
Q

What is the oldest known hereditary bleeding disorder?

A

hemophilia

48
Q

What hemophilia is “Classic Hemophilia”

A

Hemophilia A

49
Q

Hemophilia A is caused by a reduction in the amount/function of factor?

A

VIII

50
Q

What % of people with hemophilia have type A?

A

85

51
Q

Hemophilia B is also called “ _____ _____”

A

Christmas Disease

52
Q

Hemophilia B is a deficiency of factor _____

A

IX

53
Q

Both hemophilia A and B are ___ linked ___ genetic diseases?

A

X

recessive

54
Q

What disease is characterized by a prolonged bleeding time in the presence of a normal platelet count?

A

Von Willebrand’s Disease

55
Q

Patients with Von Willebrands have a defect in platelet ___ and the ______ pathway?

A
function
coagulation
56
Q

Extraoral signs of Von Willebrands include?

A

petechiae of skin

57
Q

What does Hemarthroses mean?

A

bleeding into soft tissue of joints

58
Q

What is the most common hereditary disorder of platelet function?

A

Von Willebrand’s Disease