Anemia and Red Cell Disorders-Usera Flashcards
1
Q
What is the production of RBCs in bone marrow?
A
erythropoiesis
2
Q
Erythropoiesis is stimulated by (blank) release by interstitial cells in peritubular capillaries of renal cortex
A
EPO
3
Q
What is the stimuli for EPO release?
A
Hypoexemia
Anemia
Left shift of oxygen binding curve
4
Q
How can you increase (left shift binding curve) oxygen binding affinity?
A
Increased pH
decreased DPG
decreased Temp
5
Q
How do you right shift the oxygen binding curve (i.e get rid of oxygen easily)?
A
decrease pH
increase DPG
increase temp
6
Q
What are reticulocytes a sign of?
A
active erythropoiesis
7
Q
Thread-like RNA filaments become mature RBC in (blank) hours
A
24 hours
8
Q
How is reticulocyte count recorded?
A
as a % of normal (normal less than 3%)
9
Q
What is falsely increased in anemic states?
A
reticulocyte count (must be corrrected for)
10
Q
How do you correct for reticulocyte count in anemic states?
A
Corrected Count = (actual Hct/45) x Retic count
11
Q
(blank) requires additional correction beyond this Corrected Count = (actual Hct/45) x Retic count
…. what is it?
A
Polychromasia
you divide corrected count by 2
12
Q
Extramedullary hemaotpoiesis (EMH) is naturally present in (Blank) and (Blank) -liver and spleen
A
babies and young children
13
Q
What is the compensatory response to anemia?
A
- hepatosplenomegaly
- bone marrow expansion in active marrow
14
Q
If you see accelerated erythropoiesis what should you be thinking about?
A
polycytotheia vera, anemia
15
Q
If you have hyperstimulation of bone marrow how will this present in children?
How about in adults?
A
hepatosplenomegaly
In their bones
16
Q
If you order a CBC and there is no comment on morphology what does this mean?
A
this means there is nothing wrong
17
Q
What are the measured variables provided by a CBC?
A
Hemoglobin, Hct, RBC count
18
Q
What is a calculated variable provided by a CBC?
A
RBC indices
19
Q
WHat else will a CBC provide that is not measured nor calculated?
A
WBC count with diff
Platelet count
morphology
20
Q
Newbons have (lower/higher) ranges of hemogloin thatn children and infants
A
higher
21
Q
What is this
HbF (2a2y)-shifts OBC to the left
A
fetal hemoglobin
22
Q
Over (blank) months, fetal hemoglobin cels are replaces by HbA (97%), HbA2 (2.5%) and HbF (less than 1%)
A
6-9
23
Q
Why can you get physiologic jaundice of newborn?
A
Fetal RBC with HbF are destroyed by splenic macrophages. This leads to elevations of unconjugated bilirubin.
24
Q
Children have (higher/lower) hemoglobin than adults…. why?
A
lower
Cuz they have higher phosphors levels that increase synthesis of 2,3 DPG leading to righ shifted OBC
25
Why do men have higher Hb?
due to testosterone and lack of cyclic bleeding
26
What constitutes anemia in adult male?
Hb less than 13.5 g/dL
27
What consitutes anemia in nonpregnant female?
Hb less than 12.5 g/dL
28
Why do you have lower normal levels of hemoglobin?
due to increased plasma volume resulting in dilution of hemoglobin
29
What constitutes anemia in pregnant female?
less than 11
30
What is hemoglobin electrophoresis for?
used to detect hemoglobinopathies
31
If you see abnormal structure in a hemoglobin electrophoresis what does this mean?
sickle cell anemia
32
If you see abnormal synthesis in a hemoglobin electrophoresis what does this mean?
thalassemias
33
What is the make up adult hemoglobin?
HbA 2α2ß (97% in adults)
HbA2 2α2δ (2.5% in adults)
HbF 2α2γ (1% in adults)
34
(blank) is usually a sign of an underlying disease
Anemia
35
What are the clinical findings in anemia?
```
fatigue
dyspnea
concentration difficulties
dizziness
pallor
pulmonary flow murmur
```
36
How do you find the MCV?
HCT X 1000/ RBC
37
What is MCV helpful for?
classifying anemia
38
What is considered microcytic?
less than 80
39
What is considered normocytic?
80-100
40
What is considered macrocytic?
greater than 100
41
What does MCHC stand for?
mean corpuscular hemoglobin content
42
What exactly does MCHC tell us?
what the concentration of hemoglobin is within the RBC
43
What does a bone marrow reticulocyte count less than three mean?
problems with bone marrow (cant response or hasnt responded yet)
44
A corrected reticulocyte count greater than 3 can be indicative of what?
an intrinsic RBC defect and extrinisic RBC defect
45
A reticulocyte less than three is due to what?
- Blood loss less than a week
- early-stage iron deficiency
- early-stage anemia chronic disease
- aplastic anemia
- renal disease
- malignancy
46
How do you get the MCHC (average hemoglobin concentration)?
Hb/Hct
47
What does low MCHC tell us?
implies defect in Hb synthesis; microcytic anemias
48
What does high MCHC tell us?
spherocytosis
49
(blank) is a condition that causes an abnormality in the red blood cell membrane. While healthy blood cells are shaped like flattened, indented disks, these abnormal membranes lead to sphere-shaped red blood cells, and the premature breakdown of those cells, these cells are packed with hemoglobin.
spherocytosis
50
A lot of White in the center of a RBC indicates what?
lack of hemoglobin
51
What is the red cell distribution width (RDW)?
Reflects variation in size (anisocytosis)
52
When is RDW useful?
only if it is increasd because it will indicate iron deficiency
53
What processes do RBC not do because they lack mitochondria?
No TCA
No Beta-ox
No ketone body synethesis
54
What do mature RBCs rely on for energy?
anaerobic glycolysis-cori cycle
AND
pentose phosphate pathway-synthesize glutatione
55
(blank) is a soluble iron binding storage protein
ferritin
56
serum levels of iron correlate with ferritin stores in marrow (blank)
macrophages
57
How do you get ferritin?
synthesized by macrophages and driven by IL-1 and tnf-alpha
58
(blank) ferritin is diagnostic of iron deficiency
decreased
59
Increased ferritin are signs of what?
- anemia of chronic disease
| - iron overload
60
When do you see a lot of iron often?
in people who have undergone a lot of transfusions
61
Serum iron represents iron bound to (blank)
transferrin
62
Where is transferrin synthesized?
liver
63
What is normal iron levels?
100
64
What are causes for decreased iron?
iron deficiency
| anemia chronic disease
65
What are causes of increased iron?
iron overload (transfusions, sideroblatic anemia, hemochromatosis)
66
What does TIBC stand for?
total iron-binding capacity (TIBC)
| it is the fraction of iron bound to transferrin
67
What does TIBC correlate with?
transferrin
68
What is the normal TIBC?
300
69
What is iron saturation percent?
- percentage of iron binding sites on transferrin
| - normal is 33%
70
How do you calculate iron saturation percent?
-serum iron/ TIBC x 100
71
If you are iron deficient your body wil upregulate (Blank) to try and increase iron serum levels and will decrease (blank) levels to keep from storing iron.
transferrin
| ferritin
72
What will you see in anemia chronic disease?
decreased transferrin
increased ferritin
increase hepcidin
73
Why do you see the levels of ferritin, transferrin and hepcidin that you do in anemia chronic disease?
you have chronic inflammation which will increase cytokines such as IL-1 and TNF alpha which will stimulate liver to release hepcidin and macrophages to release ferritin which tells the body you have enough iron so you decrease transferrin but since you have hepcidin you cant get the iron from ferritin to periperal blood, hence you are anemic.
74
What are the 4 microcytic anemias (less than 80)?
- iron deficiency
- anemic chronic disease
- thalassemia (a and b)
- sideroblastic anemia
75
(blank) percent of dietary iron is resorbed in duodenum
10
76
Can the oxidized (ferric Fe+) form of iron be resorbed in the duodenum?
no
77
(blank) frees elemental iron?
gastric acid
78
(blank) reduces ferric iron
Vit C
79
Most body iron is incorporated into (blank) and the remainder is stored where?
hemoglobin
| marrow macrophages, myoglobin, enzyme factors
80
Iron is absorbed in the (blank)
Folate is absorbed in the (Blank)
B12 is absorbed in the (blank)
duodenum
jejunum
colon
81
How much iron is stored in mens macrophages? Womens?
1000
| 400
82
What is the most common anemia and most common nutritional deficiency worldwide?
iron deficiency anemia
83
Toddles typically get iron defiency anemia at what age?
1-2
84
Females age (blank) tend to get iron deficiency anemia due to menstrual loss?
12-49
85
(blank) is common in nursing homes
iron deficiency anemia
86
What are some reasons that you get iron deficiency anemia due to blood loss?
```
GI bleed
Peptic Ulcer Disease
Hookworm infection
NSAIDs/Ulcers
Meckels
menorrhagia
```
87
What are some reasons that you get iron deficiency anemia due to inadequacy?
prematurity
restricted diets
malabsorption (e.g celiac)
88
What are some other reasons for iron deficiency anemi?
overutilization (pregnacy/lactation)
| Desctruction; (hemolytic anemia, PNH)
89
(blank) is a disease of the duodenum
celicas
90
What occurs in people with long term iron deficinecy anemia and what are the clinical symptoms of this?
Plummer-Vinson syndrome:
- esophageal web
- Achlohydria
- Glossitis
- koilonychia
91
What is this: fibrous constrictive ring that grows in Gi lumen and has dysphagia for solids
Esophageal web
92
What is this:
| causes red ulcers on side of mouth?
angular stomatitis
93
What is indicative of B12 deficiency?
glossitis
94
(blank) AKA spoon nailing is not specific for iron deficiency anemia but a clinical finding
Koilonychia
95
```
What will the following be like in iron deficiency anemia:
MCV?
Serum iron, iron sat?
Serum ferritin?
TIBC, RDW?
```
```
decreased
decreased
decreased
increased (cuz transferrin will be high)
(with reactive thrombocytisis)
```
96
What is the most common anemia in hospital setting?
anemia of chronic disease (ACD)
97
What are the symptoms of ACD?
- decreased synthesis of heme
- inadequate renal secretion of EPO
- hepcidin secretion by liver-response to inflammation
98
What prevents the release of iron to transferrin?
Hepcidin
99
How can chronic renal failure cause ACD?
EPO deficiency and diabetes
100
What are the lab findings for ACD:
MCV?
Serum Iron, TIBC, Iron sat?
Serum ferritin?
decreased
decreased
increased
101
What is this:
Autosomal recessive traits
Quantitative abnormality of normal globin chains
Most common in Mediterranean, Africa and southeast Asia . . .
One copy of ß gene on each chromosome 11 (2 total loci)
2 copies of α gene on each chromosome 16 (4 total loci)
Thalassemias
102
So is something wrong with your hemoglobin with thalassemias?
no, your just not making enough of it
103
Where do you commonly see thalasseias?
Mediterranean, africa, and southeast asia
104
What is the problem in beta-thalassemia?
defective upstream promotor region of beta globin gene
105
Is beta thalassemia minor macrocytic or microcytic?
microcytic
106
What do the lab values look like in beta thalassemia minor?
decreased MCV, hemoglobin and Hct
Increased RBC count
normal RDW and ferritin
107
In beta thalassemia minor you have:
(Increased/Decreased) HbA 2α2ß
(Increased/Decreased) HbA2 2α2δ and HbF 2α2γ (compensatory responses)
Decreased
| Increased
108
What is another name for beta thalassemia major?
Cooleys anemia
109
What happens in beta thalassemia major?
severe hemolytic anemia
- RBCs accumulate alpha chain inclusion, and are removed by splenic macrophages
- increased RDW and reticulocytes
110
What are the hemoglobin chains like in beta thalassemia major?
ZERO HbA 2α2ß
| Increased HbA2 2α2δ and HbF 2α2γ
111
What does beta-thalassemia major require?
long term transfusion
112
Why do you get a lot of hemolysis in beta thalassemia major?
Since your not making beta chains, you have increased alpha chaisn that fold into each other retardly and macrophages get rid of them and you have broken down RBCS so hemolytic.
113
What is the cause of alpha thalassemia?
gene deletion (one deletion does not cause anemia, its a two deletion sort of thing)
114
2 gene deletion is (blank) trait
alpha thal
115
Blacks often get what form of alpha thal? What about asians?
Blacks (α/- α/-) – mild anemia
| Asians (-/- α/α) – increased risk for more serious forms
116
What are the lab values of alph thal?
decreased MCV, Hb, Hct
Increased RBC count
Normal RDW, ferritin
117
What type of alpha thal is incompatible with life?
hydrops fetalis
118
What type of alpha thal is this:
| symptomatic, hemolytic and microcytic anemia, splenomegaly
Hb H
119
What are causes of sideroblastic anemia?
```
Chronic alcoholism
Pyridoxine deficiency (vit b6)-> common in alcoholics
Lead poisoning
Iron overload
Hereditary – x-linked recessive (rare)
```
120
What is the rate limiting step in heme formation?
delta-aminolevulinic acid
121
What is the pathogenesis of sideroblastic anemia?
-defect in heme synthesis within mitochondria,
OR
iron cannot be incorporated into heme
122
How come chronic alcoholism can cause sideroblastic anemia?
cuz ETOH is a mitochondrial toxin
123
(blank) percent of hospitalized alcohlics will have sideroblastic anemia
30
124
ALchol effects all the steps of (blank)
heme synthesis
125
What is another word for pyridoxine deficiency?
vit B6 deficiency
126
What do you use vit b6 for and if you dont have it what wil it cause?
co-factor for δ–aminolevulinic acid synthase (δ–ALA)
| the Rate-limiting step in heme synthesis, therefore causes sideroblastic anemia
127
In whom do you often see sideroblastic anemia in?
alcoholics and in isoniazid (INH) therapy
128
What do you use INH for?
TB, complexes with pyridoxine
129
Lead poisoning is the most common cause of sideroblastic anemia in whom?
little dudes 1-5 years of age cuz they eat paint AND
| battery and ammunition factory workers
130
Lead denatures critical enzymes such as ....?
ferrochelatase
ala dehydrase
ribonuclease
131
If you mess up ferrochelatase what happens?
iron cannot bind with protoporphyrin
132
What happens if you have messed up ala dehydrase?
causes increase in delta-ala
133
What happens if you have messed up ribonuclease?
prevents the breakdown of ribosomes, causing basophlic stippling
134
What are the clinical findings of sideroblastic anemia in lead poisoning?
```
Abdominal colic
Encephalopathy (due to increased δ–ALA)
growth retardation (maybe not in Tommy boy’s case though)
Peripheral neuropathy
Nephrotoxicity
Burton’s line
```
135
(blank) is toxic to neurons, causes vascular permeability and edema and demyelination
δ–ALA
136
What can you see on x rays in lead posioning?
lead deposition
137
Why do you see growth retardation in sideroblastic anemia?
lead deposits on epiphyseal plates so poor bone growth
138
What is burtons line and where do you see it?
blue line on the gingiva indicative of sideroblastic anemia
139
What are the lab findings of lead posoining (sideroblastic anemia)
Elevated lead levels (urine is the best test)
Increased iron, iron sat, and ferritin
Decreased MCV and TIBC
Ringed-sideroblasts in marrow
