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INTERNAL MEDICINE > CHAPTER 59 ANEMIA AND POLYCYTHEMIA > Flashcards

CHAPTER 59 ANEMIA AND POLYCYTHEMIA Flashcards

1
Q

Process by which the formed elements of blood are produced

A

hematopoiesis

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2
Q

what is the primary regulatory hormone for red cell production

A

epo (erythropoietin)

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3
Q

where can you find the first morphologically recognizable erythroid precursors?

A

bone marrow

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4
Q

what do you call the first morphological recognizable erythroid precursor

A

pronormoblast

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5
Q

characteristic of mature red blood cell

A

8 um in diameter
anucleate
discoid in shape
extremely pliable

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6
Q

average life span of red blood cell

A

100-120 days

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7
Q

what do you call the organ responsible for red cell production

A

erythron

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8
Q

most important factor that will affect the production of EPO

A

0xygen levels in body for tissue metabolic needs

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9
Q

where in kidney, the epo is produced?

A

peritubular capillary lining of kidney

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10
Q

do hepatocytes produce small amount of EPO?

A

true

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11
Q

what is the key to EPO gene regulation

A

hypoxia inducible factor

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12
Q

what do you call decreased red cell mass

A

anemia

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13
Q

what do you call the impaired oxygen loading of the hemoglobin

A

hypoxemia

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14
Q

what do you call the disease caused by impaired blood flow of the kidney

A

renal artery stenosis

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15
Q

what level of hemoglobin concentration that will trigger the increase of EPO levels

A

below 100-120 g/L

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16
Q

mechanism of action of EPO

A

it will bind to the specific receptors in the surface of marrow erythroid precursors inducing them to proliferate and to mature

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17
Q

effect of EPO to the red cell production

A

increase to four to five fold within 1-2 week period but only in the presence of adequate nutrients especially iron

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18
Q

laboratory result in anemia

A

hemoglobin level <130 g/L in men and <120 g/L for women

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19
Q

what are the critical elements of erythropoiesis

A

EPO production
iron availability
the proliferative capacity of the bone marrow
effective maturation of red cell precursors

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20
Q

cause of acute anemia

A

blood loss or hemolysis

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21
Q

if blood loss is mild what will happened to the curve

A

if blood loss is mid, enhanced O2 delivery is achieved thru changes in the 02 hemoglobin dissociation curved mediated by decreased ph or increased co2 (bohr effect)

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22
Q

when will the signs of vascular instability shows?

A

when there is acute losses of 10 to 15% of total blood volume

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23
Q

what will happen if there is >30% of blood volume loss

A

px unable to compensate and there will be changes in vascular flow and the px will prefer to remain supine and will show postural hypotension and tachycardia

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24
Q

if the blood is >40% what will happen to the px

A

the px will show signs of hypovolemic shock including confusion, dyspnea, diaphoresis, hypotension and tachycardia

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25
Q

how do you evaluate px with anemia

A

careful history and physical examination

nutritional history related to drugs and alcohol intake and family history of anemia should be assessed

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26
Q

what symptoms you should look for?

A 
signs of bleeding
fatigue
malaise
fever
weight loss
night sweats
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27
Q

what do splenomegaly and lymphadenopathy suggest?

A

underlying lymphoproliferative disease

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28
Q

in anemic px, physical examination will demonstrate a

A

forceful heartbeat
strong peripheral pulses
systolic flow murmur

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29
Q

where should you focus your physical examination?

A

focus on areas where vessels are close to the surface such as the mucous membrane, nail beds, palmar creases

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30
Q

when the palmar creases are lighter in color than the surrounding skin when the hand is hyperextended, what is usually the hemoglobin level

A

<80 g/L

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31
Q

physiologic factors that may affect the CBC

A 
age
sex
pregnancy
smoking
altitude
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32
Q

when can you say that it is microcytosis

A

lower MCV <80

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33
Q

When can you say it is macrocytosis

A

> 100 MCV

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34
Q

what can you get in peripheral blood smear

A

reveals the variations of cell size (anisocytosis) and shape (poikilocytosis)

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35
Q

what poikilocytosis suggests?

A

defect in the maturation of red cell precursors in the bone marrow

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36
Q

what is polychromasia

A

red cell that are slightly larger than normal and grayish-blue in color on the

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37
Q

what do you call those red cell that may appear larger and grayish in color??

A

reticulocytes

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38
Q

the color of reticulocytes represents the

A

residual amount of ribosomal rna

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39
Q

what is the key to initial classification of anemia

A

accurate reticulocyte count

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40
Q

how is reticulocytes identified?

A

by staining with supravital dye that precipitates the ribosomal RNA

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41
Q

The result of supravital dye

A

appear as blue or blank punctuate spots and can be counted manually or by fluorescent emission of dyes that bind RNA

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42
Q

To use the reticulocyte count, there is two correction necessary what are those?

A

first: adjust the reticulocyte count based on the reduced number of circulating cells
second: peripheral blood smear is examined to see if there are polychromatophilic macrocytes present

43
Q

why there is a need to correct?

A

because they are called shift cells and they survive as reticulocytes in circulation for >1 day thereby providing a falsely high estimate of daily red cell production

44
Q

what do you call the doubly corrected reticulocyte?

A

reticulocyte production index

45
Q

what laboratory measurements reflect the availability of the iron for hemoglobin synthesis?

A

serum iron
TIBC
percent transferrin saturation

46
Q

how to get the percent transferrin saturation?

A

dividing the serum iron level by the TIBC

47
Q

What is the normal serum iron range?

A

9 to 27 umol

48
Q

what is normal TIBC?

A

54 to 64 umol/L

49
Q

normal transferrin saturation

A

25 to 50%

50
Q

what is used to evaluate total body iron stores?

A

serum ferritin

51
Q

adult males have serum ferritin levels has average of ?

A

100ug/L

52
Q

adult females have serum ferritin levels at?

A

30 ug

53
Q

what serum ferritin level considered as depletion of body iron stores

A

10-15 ug

54
Q

how to check if there is an increase or decrease of one cell lineage?

A

differential count of nucleated cells in bone marrow smear

55
Q

what will be the ratio of px with hypoproliferative anemia and the reticulocyte production index?

A

M/E ratio of 2:3:1

reticulocyte index of <2

56
Q

what will be the ratio of px with hemolytic disease and reticulocyte production index?

A

ratio of 1:1

reticulocyte production index of <2

57
Q

what is the form of storage iron?

A

form of ferritin or hemosiderin

58
Q

what do you call small ferritin granules that can be seen under oil immersion in developing eryhthroblast?

A

sideroblast

59
Q

what are the three categories of functional anemia

A

marrow production defects (hypoproliferation)
red cell maturation defects (ineffective erythropoiesis)
decreased red cell survival (blood loss or hemolysis)

60
Q

what is the reticulocyte production index of hypoproliferative anemia and its cell morphology?

A

low reticulocyte index with normocytic, normochromic anemia

61
Q

what is the reticulocyte production index of maturation disorders and the cell morphology?

A

slight to moderate elevated reticulocyte production index accompanied with macrocytic or microcytic

62
Q

what the branch of anemia where the reticulocyte production index is >2.5

A

hemolysis

63
Q

examples of hemolysis or hemorrhage?

A 
blood loss
intravascular hemolysis
metabolic defect
membrane abnormality
hemoglobinopathy
immune destructions
fragmentation hemolysis
64
Q

what branch of anemia where the reticulocyte production indes is <2.5

A

red cell morphology such as hypoproliferative and maturation disorder

65
Q

what is the cell morphology in px with hypoproliferative anemia

A

normocytic normochromic

66
Q

what cell morphology is seen in px with maturation disorder?

A

micro or macrocytic

67
Q

examples of hypoproliferative?

A 
marrow damage
iron deficiency
inflammation 
metabolic disease
renal disease
68
Q

examples of maturation disorder

A

cytoplasmic defects

nuclear defects

69
Q

most cases of anemia are

A

hypoproliferative anemia

70
Q

hypoproliferative anemia reflects?

A

absolute or relative marrow failure in which degree of erythroid marrow has not proliferated appropriately

71
Q

causes of hypoproliferative anemia?

A 
marrow damage
iron deficiency
inadequate EPO stimulation
impaired renal function 
inflammation
72
Q

what are the key laboratory test in distinguishing between various form of anemia

A 
serum iron 
iron binding capacity
evaluation of renal and thyroid function
marrow biopsy
aspirate to detect marrow damage or infiltrate disease
serum ferritin
73
Q

what is used to determine the pattern of iron distribution?

A

iron stain

74
Q

what is the pattern for px with anemia of acute or chronic inflammation

A

serum iron low
TIBC normal or low
percent transferrin saturation low
serum ferritin high or normal

75
Q

what is the iron regulatory hormone that is released in the liver?

A

hepcidin

76
Q

what is the pattern in mild iron deficiency anemia

A

low serum iron
high TIBC
low percent transferrin saturation
low serum ferritin

77
Q

how to diagnose marrow damage by drugs and infiltrative disease such as leukemia or lymphoma

A

peripheral blood and bone marrow morphology

78
Q

the presence of anemia with an inappropriate low reticulocyte production index, macro-microcytes on smear and abnormal red indices suggest?

A

maturation disorder

79
Q

what is the cause of inappropriately low reticulocyte production index?

A

ineffective erythropoiesis that results from the destruction within the marrow of developing erythroblasts

80
Q

causes of nuclear maturation defects?

A

vitamin b12 or folic acid deficiency
drug damage or myelodysplasia
drugs that can cause damage DNA synthesis
alcohol

81
Q

examples of drugs that interfere with DNA synthesis

A

methotrexate or alkylating agents

82
Q

causes of cytoplasmic maturation defects?

A

severe iron deficiency

abnormalities in globin or heme synthesis

83
Q

acute blood loss is not associated with an increased reticulocyte production index because of the time required to increase EPO production and subsequently marrow proliferation

A

true

84
Q

what is the most important step before initiating treatment for anemia?

A

evalutate the px iron stores status before and during the treatment of any anemia

85
Q

what is the most important step before initiating treatment for anemia?

A

evalutate the px iron stores status before and during the treatment of any anemia

86
Q

what is the treatment for px with anemia with chronic renal failure and in dialysis

A

recombinant EPO

87
Q

What is the treatment for px with inherited genetic conditions?

A

targeted genetic therapy

88
Q

it is defined as increased in the hemoglobin above normal

A

polycythemia

89
Q

the difference between erythrocytosis and polycythemia

A

erythrocytosis-documentation increased red cell mass

polycythemia-any increase in red cell

90
Q

hematocrit level considered as abnormal

A

> 50 percent for men

>45 for women

91
Q

features of clinical history that are useful for differentials?

A 
smoking history
current living at high altitude
history of diuretic use
congenital heart disease
sleep apnea
chronic lung disease
92
Q

classic symptoms in polycythemia

A

hyperviscosity and thrombosis both venous and arterial

neurologic symptoms

93
Q

symptoms of polycythemia vera

A 
aquagenic pruritus
hepatosplenomegaly
easy bruising
epistaxis
peptic ulcer
94
Q

p.e in px with polycythemia vera

A

ruddy complexion

95
Q

the presence of cyanosis or evidence of left to right shunt suggest?

A

congenital heart disease such as tetralogy of fallot or Eisenmenger syndrome

96
Q

increased blood viscosity can raise pulmonary artery pressure while hypoxemia can lead to increased pulmonary vascular resistance can lead to?

A

cor pulmonale

97
Q

how to document polycythemia using the principle of isotope dilution

A

use Cr-labeled autologous red blood cells to the px and sampling blood radioactivity for 2 hours

98
Q

if the result of dilution normal, the px has?

A

spurious or relative polycythemia

99
Q

if the result of the dilution is high red cell mass, what should be done?

A

measure epo and if the epo is low then the px has polycythemia vera

100
Q

and if the EPO is elevated?

A

distinguish whether elevation is a physiologic response to hypoxia or related to autonomous EPO production

101
Q

why is it that smokers has normal o2 saturation but have elevated EPO levels

A

because of CO displacement ofO2

102
Q

if the carboxyhemoglobin levels are high what should be the diagnosis?

A

smoker’s polycythemia

103
Q

what is the treatment for smoker’s polycythemia

A

stop smoking

if not then they require phlebotomy to control their polycythemia

INTERNAL MEDICINE (12 decks)

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