Exam 1 Material Flashcards

(109 cards)

1
Q

What are the 4 Pathophysiological categories of anemia?

A

1) Whole blood loss from the vasculature–
2) Increased destruction of red cells

3) Decreased production of red cells
4) Anemia of pregnancy

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2
Q

Describe the pathogenesis of whole blood loss from the vascular system.

A

Can be–

a) acute
b) chronic

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3
Q

Describe the pathogenesis of increased destruction of red cells.

A

a) Maybe acquired such as hemolytic transfusion reaction or malarial parasites.

B) Maybe due to intracellular defects which are hereditary or congenital such as enzyme deficiencies, unstable cell membranes, or abnormal hemoglobin molecules.

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4
Q

Describe the pathogenesis of decreased production of red cells

A

Can be due to–

A) a defective stem cell

B) deficiencies of necessary materials for cell production such as iron, porphyrins, or globin

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5
Q

Name three sites where hemolysis may occur leading to anemia

A

A) intravascular

B) extravascular such as the liver and spleen

C) intramedullary

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6
Q

What is the pathophysiological classification of iron deficiency anemia?

A

An anemia of decreased production

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7
Q

What is the etiology of iron deficiency anemia?

A

A) nutritional as in decreased intake

B) increased need when menstruating, pregnant, or lactating or growing children and teens

C) increased loss such as chronic blood loss

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8
Q

In what order do the iron compartments become depleted?

A

A) Storage

B) Transport

C) Functional

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9
Q

Which laboratory indice will show a change in value first during IDA?

A

Serum ferritin will be decreased first

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10
Q

In addition to generic signs of anemia, what other signs are observed in IDA?

A

Pica
Glossitis
Angular Chelosis
Koilonychia

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11
Q

What will the Laboratory test values be in Stage 1 iron depletion?

A

Hemoglobin –> Normal
Serum Iron –> Normal
TIBC –> Normal
Serum Ferritin –> Decreased

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12
Q

What will the Laboratory test values be in Stage 2 iron depletion?

A

Hemoglobin –> Normal
Serum Iron –> Decreased
TIBC –> Increased
Serum Ferritin –> Decreased

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13
Q

What will the laboratory test values be in Stage 3 Iron Depletion? (IDA)

A

Hemoglobin –> Decreased
Serum Iron –> Decreased
TIBC –> Increased
Serum Ferritin –> Decreased

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14
Q

What is the Pathophysiological classification of IDA?

A

Anemia of decreased production

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15
Q

What is the Wintrobe classification of IDA?

A

Microcytic

Hypochromic

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16
Q

What will the RDW be in IDA?

A

Increased

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17
Q

What will the RBC count be in IDA?

A

Increased
Decreased
Normal

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18
Q

Will you see thrombocytosis in IDA?

A

May

or May Not

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19
Q

Are WBC affected in IDA?

A

Typically Not

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20
Q

Will you see Polychromasia in IDA?

A

Early, yes

But retic index <3%

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21
Q

What significance does measuring serum ferritin have?

A

Gives a reflection of the iron stores of the marrow

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22
Q

How is ferritin measured?

A

Immunoassay

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23
Q

What are two acute phase reactants?

A

Hepcidin

Ferritin

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24
Q

What is the Ref Range of ferritin?

A

20-250 ug/L

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25
When and why can ferritin levels be misleadingly high?
During inflammation because ferritin is an acute phase reactant!!
26
How is Total Serum Iron measured?
Iron released from transferrin using acid | Measured spectrophotometrically by reacting with ferrozine
27
What is the Ref Range of Total Serum Iron?
70-201 ug/dL | 12.7-35.9 umol/L
28
How is the TIBC measured?
Iron is added to serum to saturate transferrin Excess iron removed Remove iron from transferrin with acid React with ferrozine and measure spectrophotometrically Total serum Iron subtracted from amount measured
29
What is the Ref Range for TIBC?
45.2-77.7 umol/L | 253-435 ug/dL
30
How do you calculate % Saturation of transferrin?
Serum Iron/TIBC X 100%
31
What are the Ref Ranges for %Sat in women and men?
``` Women = 15-50% Men = 20-50% ```
32
How is Free Erythrocyte Protoporphyrin measured?
Fluorometry
33
What is the unit of FEP?
mmol PP/mol heme
34
What is zinc protoporhyrin?
Protoporphyrin with zinc instead of iron. | If no iron is present, zinc can be incorporated instead
35
How is ZPP measured?
Fluorometry
36
What is the unit of ZPP?
mmol ZPP/mol heme
37
How is serum transferrin receptor measured?
immunoassay
38
What is the Ref Range of Serum Transferrin Receptor?
4.54-6.18 mg/L
39
What does CHr stand for?
Reticulocyte Hemoglobin content
40
What is the value of the CHr?
Reflects the iron of the BM within 1 day!!
41
What is the value of the CHr when a patient is iron deficient?
CHr <28%
42
What is the Treatment of IDA?
Iron Supplements--> for months beyond no sign of anemia | Eliminate sources of loss
43
What is the pathophysiological classification of ACI?
Anemia of decreased production
44
What is the etiology of ACI?
Associated with chronic inflammation A) Autoimmune diseases B) Chronic infections--> TB, Syphilis C) Malignancies/Chronic debilitating diseases-->Heart disease
45
What term describes low serum iron?
Sideropenia
46
What cytokines are produced by activated macrophages?
TNF IL-1 B interferon Gamma Interferon
47
What is the Hgb level in a patient with ACI?
Usually >or = 10g/dL
48
What is the RDW in ACI?
Usually Normal
49
Are WBC/Plts affected in ACI?
NO
50
What is the Absolute Retic Count in ACI?
Decreased
51
What is the ferritin in ACI?
Normal or increased
52
What is the serum iron in ACI?
Decreased
53
What is the TIBC in ACI?
Decreased
54
What is the %Sat in ACI?
Normal or decreased
55
What is the FEP/ZPP in ACI?
Increased
56
What is the amount of stainable iron in the marrow in ACI?
Normal or increased
57
What is the level of Soluble transferrin receptor in ACI?
Normal
58
What is the CHr in ACI?
Normal
59
What is the treatment for ACI?
Cure the underlying cause of inflammation! Typically not so dramatic as to require any treatment--> remember, Hgb is 10 or greater!! Give Iron IV--> Remember, enterocytes are not absorbing iron due to increase in Hepcidin, so give it IV!
60
What is the pathophysiological classification of Sideroblastic anemia?
Anemia of decreased production | can have a hemolytic component too
61
What process is impaired in Siderblastic anemia?
Heme synthesis
62
What is the etiology of Sideroblastic Anemia?
Hereditary Acquired a) Drugs such as chloramphenicol b) Heavy metals such as Pb and Hg
63
Why do we see a buildup of ALA and PPIX in Sideroblastic Anemia?
Lead impairs heme synthesis at several steps, in particular: a) ALA-Dehydratase b) Ferrochelatase
64
What are the clinical findings in children and adults with lead poisoning?
Adults with acute toxicity--> Nausea, vomiting, headache Adults with chronic toxicity--> Neuropathies, Abdominal Cramping, Seizures Children--> Similar to adults, may see encephalopathy which can lead to convulsions and mental impairments
65
What do the RBC's look like in ACI?
Normochromic, Normocytic Can be microcytic!!
66
What do the RBC's look like in Siderblastic Anemia?
Normocytic, Normochromic Can be Hypochromic, Microcytic!!!
67
What is the RDW in Siderblastic Anemia?
WRR
68
What RBC inclusions will be observed in Sideroblastic Anemia?
Basophilic Stippling | Pappenheimer bodies
69
Are WBC/Plts affected in Siderblastic Anemia?
NO
70
What is the reticuloctye count in Sideroblastic Anemia?
Normal or Increased if the hemolytic component is significant
71
What will you see in the BM of a patient with Siderblastic Anemia?
Ringed Sideroblast!!!!! Because cell cannot incorporate iron into heme which occurs in the mitochondria. Iron appears in the mitochondria forming a ring around nucleus
72
What will the ferritin be in Siderblastic Anemia?
Normal
73
What will the serum iron be in Siderblastic Anemia?
Normal or increased
74
What is the TIBC in Sideroblastic Anemia?
Normal
75
What is the %Sat in Sideroblastic Anemia?
Normal or increased
76
What is the FEP/ZPP in Sideroblastic Anemia?
Greatly Increased
77
What is the soluble transferrin receptor in Sideroblastic Anemia?
Normal
78
What is the CHr in Sideroblastic Anemia?
Normal?
79
What is the treatment of Sideroblastic Anemia?
Eliminate Lead | Chelation therapy using EDTA--> removes lead for excretion in the URN
80
What is the pathophysiological classification of thalassemia?
Anemia of increased destruction
81
What are the clinical symptoms of thalassemia?
Chronic Anemia Splenomegaly Bone Abnormalities Gall Stones made of bilirubin
82
What genetic mutation causes B+?
Point mutation in the promoter region leading to decreased binding of RNA polymerase. Get some transcription, and subsequently some translation. Net result--> Get some normal globin chains, but low level
83
What genetic mutation causes B0?
Chain terminator mutation where stop codon is inserted. Leads to non-functional globin fragments. Net result--> No B chain production
84
What genetic mutation can result in either B0 or B+?
Splicing Mutation which can affect normal splice site or create a new one. Net result--> non-functional fragments or non-functional extensions at splice site
85
What do the RBC's look like in thalassemia?
Microcytic, Hypochromic Will see Target Cells, ovalocytes, schistocytes Basostippling
86
What is the RDW in thalassemia?
Normal or increased
87
What is the reticulocyte index in thalassemia?
Reticulocytosis but insufficient to compensate for the anemia
88
What is the serum iron in thalassemia?
Normal or increased
89
What is the TIBC in thalassemia?
Normal
90
What is the %Sat in thalassemia?
Normal or increased
91
What is the ferritin in thalassemia?
Normal or increased
92
What is the FEP in thalassemia?
Normal
93
What is the iron level in BM in thalassemia?
Increased
94
What is the treatment of thalassemia?
Transfusion--> Results in hemosiderosis, so must undergo chelation therapy to excrete excess iron into urn. Increased iron deposits can cause organ damage due to oxidative damage by iron. Bone Marrow transplant
95
What is the pathophysiological classification of Megaloblastic Anemia?
Anemia of decreased production
96
What is the etiology of Megaloblastic Anemia?
Deficiency of Vitamin B12 or Folate
97
How is Vit B12 carried in the plasma?
Haptocorrin--> Not metabolically active--> Cells do not have receptor for it Transcobalamin II--> Metabolically active
98
In what ways can a patient have impaired absorption of Vit B12?
Chronic Pancreatic Disease causing decrease in Trypsin Chronic Bowel Disease leading to maladsorption Competition form Diphyllobothrium datum Intrinsic Factor Deficiency
99
What are the clinical signs of Vit B12 deficiency?
Peripheral Neuropathy affecting the gait Glossitis Gastrointestinal Distress
100
What do the RBC's look like in Megaloblastic Anemia?
Macrocytic, Normochromic
101
Will see pancytopenia in Megaloblastic Anemia!!
A lack of Vit B12 or Folate limits DNA synthesis and ultimately cell division.
102
What is the RDW in Megaloblastic Anemia?
Increased
103
What is considered pathognomonic for Megaloblastic Anemia?
Hypersegmented Neutrophils!!!
104
What Red Cell inclusions may be observed with Megaloblastic Anemia?
Nucleated RBC Howell-Jollly Bodies Basophilic Stippling
105
What is the reticulocyte count in Megaloblastic Anemia?
LOW
106
What is different about the maturation of cells in Megaloblastic Anemia?
The nucleus lags behind the cytoplasm
107
What is the treatment of Megaloblastic Anemia?
Eliminate underlying cause Supplement missing Vitamin Supplement with iron because red cell production is very quick!!
108
What is the pathophysiological classification of Myelodysplasia?
Anemia on decreased production due to a mutated stem cell
109
What will the RBC's look like in uncomplicated alcoholism?
Macrocytic, Normochromic Target Cells with or without acanthocytes