chapter 12 - hematological disorders Flashcards

1
Q

what are the two components of blood

A

plasma and cellular components (RBC, WBC, Platelets)

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2
Q

what is in plasma

A

water, proteins, carbs, lipids, electrolytes, clotting factors

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3
Q

what do platelets and coagulation factors in plasma do

A

maintain homeostasis

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4
Q

what is the principal site of blood cell formation

A

bone marrow

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5
Q

what is hematopoiesis

A

production of blood cells

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6
Q

what protein regulates RBC production and where is it produced

A

erythropoietin, in the kidney

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7
Q

what is the main function of RBC and what is the carrier substance

A

transport of oxygen to the tissues, hemoglobin is the carrier substance

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8
Q

what is the life span of a normal RBC and what s the process of removing them

A

120 days

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9
Q

what is the term for increased WBC

A

leukocytosis

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10
Q

what is the term for decreased WBC

A

leukopenia

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11
Q

what are some causes for leukocytosis

A

acute bacterial infection, physical stimuli (heat, cold, pain, surgery, malignancies, drug reactions), emotional stimuli (stress)

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12
Q

when is leukopenia often seen

A

with viral or overwhelming bacterial infection

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13
Q

what is the term for increased RBC

A

polycythemia

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14
Q

microcytic cells are seen in ______ (conditions)

A

iron def anemia and thalessemia

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15
Q

macrocystic cells re seen in ___(conditions)

A

liver disorders, alcoholism, folic acid, vit b12 deficiencies

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16
Q

what is MCV describing

A

RBC size (microcytic or macrocytic)

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17
Q

what is MCH

A

average weight of hemoglobin in each individual RBC

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18
Q

what does MCHC measure

A

average weight of hemoglobin in each individual RBC

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19
Q

what are normocytes

A

normal sized erythrocytes

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20
Q

what is anisocytosis

A

abnormal variation in cell size

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21
Q

what is poikilocytosis

A

variation in RBC shape

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22
Q

what values should be analyzed in anemia

A

hemoglobin, MCV and MCH

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23
Q

what values are used to classify anemia as normocytic, microcytic or macrocytic, or normochronic or hypochromic

A

MCV and MCH

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24
Q

anemias with normal MCV and MCH are called _______ and __________

A

normocytic and normochromic

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25
Q

anemia of chronic blood loss, chronic disease and aplastic anemia are _____

A

normocytic and normochromic

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26
Q

anemias with decreased MCV and MCH are

A

microcytic and hypochromic

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27
Q

decreased MCV and MCH are almost always seen with which type of anemia

A

iron deficient anemia

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28
Q

very low MCV is common seen with ____

A

thalassemia

29
Q

anemias with elev MCV are ______ and can be due to _______

A

macrocytic, due to folic acid deficiency, b2 def, or a problem with intrinsic factor that transports fit b12 ro the intestine

30
Q

what other thing can elev MCV be due to

A

excess alcohol intake

31
Q

describe RBC in macrocytic anemia

A

fewer but larger RBC

32
Q

what is megaloblastic anemia

A

imprecise term that designates a group of anemias having similar characteristics of morphology and function

33
Q

what is aplastic anemia

A

a bone marrow disorder characterized by a reduction of hemopoietic tissue, replacement of hemopoietic tissue, and depletion of all cell lines (pancytopenia)

34
Q

what is pure red cell aplasia

A

like aplastic anemia but only the RBC precursors are hypoplastic

35
Q

does aplasia anemia have poor prognosis

A

yes

36
Q

what is the overall mortality rate for aplastic anemia and the median survival

A

65-70%, 3 months

37
Q

what is the most common cause of death with aplastic anemia

A

hemorrhage or infection

38
Q

what is the most favorable situation re aplastic anemia for underwriting

A

cause clearly diagnosed and anemia resolved for several years

39
Q

what is a hemolytic process

A

shortened RBC survival

40
Q

what is hereditary sperhocytosis and how is it treated

A

a hemolytic anemia with sphere shaped RBC and destroyed in the spleen. treated with splenectomy

41
Q

children who have splenectomy have higher mortality until what age

A

15-18

42
Q

what is G-6-PD deficiency

A

glucose-6-phosphate dehydrogenase def, hereditary abnormal in which the activity or stability of the enzyme G-6-PD is markedly diminished

43
Q

sickle cell disease is often fatal before age ___

A

30

44
Q

the anemia of thalassemia is due to ____

A

both decreased RBD production and hemolysis

45
Q

what is the homozygous form of thalassemia

A

thalassemia major

46
Q

what is the heterozygous form of thalassemia

A

thalassemia minor

47
Q

what is polycythemia vera

A

myeloproliferative disorder of the bone marrow that can terminate in acute granulocytic leukemia

48
Q

in polycythemia vera what is increased

A

RBC, hemoglobin, hematocrit, RBC mass and viscosity of blood

49
Q

approx 30% of patients with polycythemia vera will develop ____

A

leukemia

50
Q

what is treatment for polycythemia vera

A

period blood draws (phlebotomy)

51
Q

which type of WBC are the most numerous

A

neutrophils

52
Q

which type of WBC is most important in fighting bacterial infections and inflammation

A

neutrophils

53
Q

which type of WBC has a strong association with allergies

A

eosinophilic

54
Q

which type of WBC is rare and carries heparin and histamines

A

basophils

55
Q

what is he second most abundant WBC

A

lymphocytes

56
Q

what are monocytes

A

the largest cell of normal blood, ingests and destroys particles, debris, and bacteria

57
Q

is hemophilia A seen more in males or females

A

males

58
Q

is Von Willebrands seen more in males or females

A

females

59
Q

hemophilia A is caused by a deficiency of

A

factor VIII

60
Q

what is another name for hemophilia B

A

christmas disease

61
Q

hemophilia B is caused by a deficiency of

A

factor IV

62
Q

Von willebrands involves

A

platelet and factor VIII deficiencies

63
Q

aplastic anemia is characterized by all of the following EXCEPT:

  1. replacement of hemopoietic tissue
  2. depletion of cell lines
  3. reduction of hemopoietic tissue
  4. hypercellular bone marrow
A
  1. hypercellular bone marrow
64
Q

in polycythemia vera, are hemoglobin and hematocrit usually decreased at diagnosis?

A

no

65
Q

what is the malignant disorder characterized by the uncontrolled proliferation of abnormal WBC

A

leukemia

66
Q

Is hemophilia a also known as Christmas disease

A

No

67
Q

Is hemophilia a caused by a factor IX deficiency

A

No

68
Q

Does hemophilia a developed predominantly in males or females

A

Males

69
Q

What is decreased synthesis of one of the polypeptide chains that comprise hemoglobin

A

Thalasssmia