CHAPTER 59 ANEMIA AND POLYCYTHEMIA Flashcards
(103 cards)
1
Q
Process by which the formed elements of blood are produced
A
hematopoiesis
2
Q
what is the primary regulatory hormone for red cell production
A
epo (erythropoietin)
3
Q
where can you find the first morphologically recognizable erythroid precursors?
A
bone marrow
4
Q
what do you call the first morphological recognizable erythroid precursor
A
pronormoblast
5
Q
characteristic of mature red blood cell
A
8 um in diameter
anucleate
discoid in shape
extremely pliable
6
Q
average life span of red blood cell
A
100-120 days
7
Q
what do you call the organ responsible for red cell production
A
erythron
8
Q
most important factor that will affect the production of EPO
A
0xygen levels in body for tissue metabolic needs
9
Q
where in kidney, the epo is produced?
A
peritubular capillary lining of kidney
10
Q
do hepatocytes produce small amount of EPO?
A
true
11
Q
what is the key to EPO gene regulation
A
hypoxia inducible factor
12
Q
what do you call decreased red cell mass
A
anemia
13
Q
what do you call the impaired oxygen loading of the hemoglobin
A
hypoxemia
14
Q
what do you call the disease caused by impaired blood flow of the kidney
A
renal artery stenosis
15
Q
what level of hemoglobin concentration that will trigger the increase of EPO levels
A
below 100-120 g/L
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
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
18
Q
laboratory result in anemia
A
hemoglobin level <130 g/L in men and <120 g/L for women
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
20
Q
cause of acute anemia
A
blood loss or hemolysis
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)
22
Q
when will the signs of vascular instability shows?
A
when there is acute losses of 10 to 15% of total blood volume
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
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
25
how do you evaluate px with anemia
careful history and physical examination
| nutritional history related to drugs and alcohol intake and family history of anemia should be assessed
26
what symptoms you should look for?
```
signs of bleeding
fatigue
malaise
fever
weight loss
night sweats
```
27
what do splenomegaly and lymphadenopathy suggest?
underlying lymphoproliferative disease
28
in anemic px, physical examination will demonstrate a
forceful heartbeat
strong peripheral pulses
systolic flow murmur
29
where should you focus your physical examination?
focus on areas where vessels are close to the surface such as the mucous membrane, nail beds, palmar creases
30
when the palmar creases are lighter in color than the surrounding skin when the hand is hyperextended, what is usually the hemoglobin level
<80 g/L
31
physiologic factors that may affect the CBC
```
age
sex
pregnancy
smoking
altitude
```
32
when can you say that it is microcytosis
lower MCV <80
33
When can you say it is macrocytosis
>100 MCV
34
what can you get in peripheral blood smear
reveals the variations of cell size (anisocytosis) and shape (poikilocytosis)
35
what poikilocytosis suggests?
defect in the maturation of red cell precursors in the bone marrow
36
what is polychromasia
red cell that are slightly larger than normal and grayish-blue in color on the
37
what do you call those red cell that may appear larger and grayish in color??
reticulocytes
38
the color of reticulocytes represents the
residual amount of ribosomal rna
39
what is the key to initial classification of anemia
accurate reticulocyte count
40
how is reticulocytes identified?
by staining with supravital dye that precipitates the ribosomal RNA
41
The result of supravital dye
appear as blue or blank punctuate spots and can be counted manually or by fluorescent emission of dyes that bind RNA
42
To use the reticulocyte count, there is two correction necessary what are those?
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
why there is a need to correct?
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
what do you call the doubly corrected reticulocyte?
reticulocyte production index
45
what laboratory measurements reflect the availability of the iron for hemoglobin synthesis?
serum iron
TIBC
percent transferrin saturation
46
how to get the percent transferrin saturation?
dividing the serum iron level by the TIBC
47
What is the normal serum iron range?
9 to 27 umol
48
what is normal TIBC?
54 to 64 umol/L
49
normal transferrin saturation
25 to 50%
50
what is used to evaluate total body iron stores?
serum ferritin
51
adult males have serum ferritin levels has average of ?
100ug/L
52
adult females have serum ferritin levels at?
30 ug
53
what serum ferritin level considered as depletion of body iron stores
10-15 ug
54
how to check if there is an increase or decrease of one cell lineage?
differential count of nucleated cells in bone marrow smear
55
what will be the ratio of px with hypoproliferative anemia and the reticulocyte production index?
M/E ratio of 2:3:1
| reticulocyte index of <2
56
what will be the ratio of px with hemolytic disease and reticulocyte production index?
ratio of 1:1
| reticulocyte production index of <2
57
what is the form of storage iron?
form of ferritin or hemosiderin
58
what do you call small ferritin granules that can be seen under oil immersion in developing eryhthroblast?
sideroblast
59
what are the three categories of functional anemia
marrow production defects (hypoproliferation)
red cell maturation defects (ineffective erythropoiesis)
decreased red cell survival (blood loss or hemolysis)
60
what is the reticulocyte production index of hypoproliferative anemia and its cell morphology?
low reticulocyte index with normocytic, normochromic anemia
61
what is the reticulocyte production index of maturation disorders and the cell morphology?
slight to moderate elevated reticulocyte production index accompanied with macrocytic or microcytic
62
what the branch of anemia where the reticulocyte production index is >2.5
hemolysis
63
examples of hemolysis or hemorrhage?
```
blood loss
intravascular hemolysis
metabolic defect
membrane abnormality
hemoglobinopathy
immune destructions
fragmentation hemolysis
```
64
what branch of anemia where the reticulocyte production indes is <2.5
red cell morphology such as hypoproliferative and maturation disorder
65
what is the cell morphology in px with hypoproliferative anemia
normocytic normochromic
66
what cell morphology is seen in px with maturation disorder?
micro or macrocytic
67
examples of hypoproliferative?
```
marrow damage
iron deficiency
inflammation
metabolic disease
renal disease
```
68
examples of maturation disorder
cytoplasmic defects
| nuclear defects
69
most cases of anemia are
hypoproliferative anemia
70
hypoproliferative anemia reflects?
absolute or relative marrow failure in which degree of erythroid marrow has not proliferated appropriately
71
causes of hypoproliferative anemia?
```
marrow damage
iron deficiency
inadequate EPO stimulation
impaired renal function
inflammation
```
72
what are the key laboratory test in distinguishing between various form of anemia
```
serum iron
iron binding capacity
evaluation of renal and thyroid function
marrow biopsy
aspirate to detect marrow damage or infiltrate disease
serum ferritin
```
73
what is used to determine the pattern of iron distribution?
iron stain
74
what is the pattern for px with anemia of acute or chronic inflammation
serum iron low
TIBC normal or low
percent transferrin saturation low
serum ferritin high or normal
75
what is the iron regulatory hormone that is released in the liver?
hepcidin
76
what is the pattern in mild iron deficiency anemia
low serum iron
high TIBC
low percent transferrin saturation
low serum ferritin
77
how to diagnose marrow damage by drugs and infiltrative disease such as leukemia or lymphoma
peripheral blood and bone marrow morphology
78
the presence of anemia with an inappropriate low reticulocyte production index, macro-microcytes on smear and abnormal red indices suggest?
maturation disorder
79
what is the cause of inappropriately low reticulocyte production index?
ineffective erythropoiesis that results from the destruction within the marrow of developing erythroblasts
80
causes of nuclear maturation defects?
vitamin b12 or folic acid deficiency
drug damage or myelodysplasia
drugs that can cause damage DNA synthesis
alcohol
81
examples of drugs that interfere with DNA synthesis
methotrexate or alkylating agents
82
causes of cytoplasmic maturation defects?
severe iron deficiency
| abnormalities in globin or heme synthesis
83
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
true
84
what is the most important step before initiating treatment for anemia?
evalutate the px iron stores status before and during the treatment of any anemia
85
what is the most important step before initiating treatment for anemia?
evalutate the px iron stores status before and during the treatment of any anemia
86
what is the treatment for px with anemia with chronic renal failure and in dialysis
recombinant EPO
87
What is the treatment for px with inherited genetic conditions?
targeted genetic therapy
88
it is defined as increased in the hemoglobin above normal
polycythemia
89
the difference between erythrocytosis and polycythemia
erythrocytosis-documentation increased red cell mass
| polycythemia-any increase in red cell
90
hematocrit level considered as abnormal
>50 percent for men
| >45 for women
91
features of clinical history that are useful for differentials?
```
smoking history
current living at high altitude
history of diuretic use
congenital heart disease
sleep apnea
chronic lung disease
```
92
classic symptoms in polycythemia
hyperviscosity and thrombosis both venous and arterial
| neurologic symptoms
93
symptoms of polycythemia vera
```
aquagenic pruritus
hepatosplenomegaly
easy bruising
epistaxis
peptic ulcer
```
94
p.e in px with polycythemia vera
ruddy complexion
95
the presence of cyanosis or evidence of left to right shunt suggest?
congenital heart disease such as tetralogy of fallot or Eisenmenger syndrome
96
increased blood viscosity can raise pulmonary artery pressure while hypoxemia can lead to increased pulmonary vascular resistance can lead to?
cor pulmonale
97
how to document polycythemia using the principle of isotope dilution
use Cr-labeled autologous red blood cells to the px and sampling blood radioactivity for 2 hours
98
if the result of dilution normal, the px has?
spurious or relative polycythemia
99
if the result of the dilution is high red cell mass, what should be done?
measure epo and if the epo is low then the px has polycythemia vera
100
and if the EPO is elevated?
distinguish whether elevation is a physiologic response to hypoxia or related to autonomous EPO production
101
why is it that smokers has normal o2 saturation but have elevated EPO levels
because of CO displacement ofO2
102
if the carboxyhemoglobin levels are high what should be the diagnosis?
smoker's polycythemia
103
what is the treatment for smoker's polycythemia
stop smoking
| if not then they require phlebotomy to control their polycythemia
