Week 5 lab diagnosis lecture Flashcards

(98 cards)

1
Q

main function of RBC?

A

O2 from lung to tissues, CO2 from tissues to lung

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2
Q

what enables RBC to move O2

A

Hemoglobin

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3
Q

Structure of hemoglobin

A

tetromer with heme in each subunit

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4
Q

what is each polypeptide of hemoglobin called?

A

a globin, 2 alphas and 2 betas

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5
Q

what part of globbin attracts oxygen?

A

Iron in the porforin containing heme structure

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6
Q

what is heme

A

Metallo-porphoryin that contains iron, porphoryin and binds respiratory gasses

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7
Q

What is globin

A

a protein of 4 aa chains , alpha and beta

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8
Q

what makes up a hemoglobin

A

4 globins with their 4 hemes

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9
Q

two states of hemoglobin

A

oxyhemoblobin when bound to oxygen and deoxyhemoglobin without oxygen.

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10
Q

Color of deoxyhemoglobin

A

blusih red rather than bright red where oxygen is bound

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11
Q

what are the 3 normal variations of hemoglobin?

A

Hemoglobin A, A2, and F.

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12
Q

Hemoglobin A

A

97%, 2 alpha and 2 beta chains

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13
Q

Hemoglobin A2

A

Less than 3%, 2 alpha and 2 delta chains

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14
Q

Hemoglobin F

A

1%, 2 alpha and 2 gamma chains, fetal developmental

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15
Q

so what chains are in all 3 normal variaitons of hemoglobin

A

there are always two alpha chains

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16
Q

When is Hemaglobin F abundant?

A

F is for fetal, so at birth its 50-80% that is down to 8 % in 6 months then 1-2 % into adulthood

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17
Q

what is a hemoglobinopathies

A

family of genetic disorders that have structurally abnormal or insufficient quanities of hemoglobin or both

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18
Q

what is a qualitative hemoglobinopathie

A

producing structrual abnormal hemoglobin

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19
Q

what is a quantative hemoglobinopathies

A

producing insufficient quanities of normal hemoglobin.

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20
Q

What is thalassemia?

A

Genetic blood disorder where body makes reduced numbers of globin chains

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21
Q

what are the two types of thalassemia?

A

Alpha thalassemia and beta thalassemia

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22
Q

Where is alpah thalassemia most common,

A

SE asia, middle east and china effecting alpha chains

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23
Q

Where is beta thalassemia most common

A

mediterranean affecting beta globins

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24
Q

what are the forms of alpha and beta thalassemia

A

both major and minor

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25
symptoms of major thalassemia
anemia in first year, jaundice, facial bone deformities, growth failure and shortness of breath
26
what would blood from Beta thalassemia major look like?
poikilocytic RBC, so lots of different shapes
27
what is the most common form of beta thalassemia
Beta thalassemia minor where only one beta chain is affected
28
Beta thalassema minor looks like what?
mild microcytic hypochromic anemia with no other clinical symptioms., so RCBs are smal with less color
29
what is hemoglobin S (sickel) ?
Predominant in those with sickel sell, variation on beta chain
30
Hemoglobin C?
disease with mild hemolytic anemia and splenomegaly
31
Hemoglobin E?
Mild hemolytic anema and splenomegaly, very common in SE asia
32
Hemoglobin constan spring
alpha chain is abnormally long resulting in thalassemic phenotype.
33
Hemoglobin H?
made of 4 beta chains because Alphas arnt made
34
Hemoglobin barts
No alpha chain produced, die in utero moslty
35
Hemoglobinopathies
inherited defects in globin structure mostly involving a single aa substitution
36
what makes sickle cells change shape
Glutamic acid instead of valine allows hemoglobin to polymerize, link inot long stiff structure that stretches out RBC
37
How long does sickel cell last?
20 or less days, its also rigid
38
Baso-occlusion
sickle cells block blood to microscopic regions of tissue because of their shape and regidity, this causes hypoxia of tissued, pain and organ damage
39
what % has sickel cell trait, but are heterozygous
8 % of african americans, without evidence of dz mostly, may have splenic infarcts if hypoxia or hematuria
40
For heterozygous sickel what is the % of Hgb S?
20-40% of hemoglobin is Hgb S
41
For homozygous sickle cell, what % of S hemoglobin
80-100 %
42
what % has sickle cell disease
less than 1 % of african americans
43
when does sickle cell disease show?
after 6 months, then show anema with slight jaundice
44
symptoms of sickel cell?
die before 40, infarcts in various organs and bone pain
45
What is hemoglobin D disease
similar to HbS but lysine is substatuted in, causing milded hemolytic andmia
46
what happens to RBCs in hemoglobin D instead of shape change
precipitation of hemoglobin crytalize, so they get removed by spleen, reducing risk for malaria
47
Hemoglobin C dz. Who has it?
3% of African american from Africa
48
what does Hb C dz do?
reduction of RBC lifespan, abdominal and bone pain, large number of target cells, less severe thatn sickle dz
49
why may HbS reduce malaria?
reduce oxygen tension in cells retarding parasite growth
50
why may A thalassema reduce malaria?
if gets mild malaria is now immune to more severe form
51
Why B thalassema reduce malaria?
?, maybe reduction in cell adhesion in cerebral form of malaria
52
where does the heme synthesis happen
85% in RBC but it quits when they mature
53
where does the rest of the heme synthesis happen
mostly the liver, its used for synthesis of cytochrome p450 enzymes for detox
54
what are Porphorias?
dz that are caused by deficiencies of enzymes of the heme biosynthetic pathy
55
what are the two main porphorias?
Acute intermetted porhyria, porphyria cutanea darda
56
what are the two cardinal symptoms of those with porphorya
Photosensitivity and neurologic disorders
57
Acute porphyrias
affect NS, quickly start and end, abdominal pain, vomiting, diarrhea, constipation, hallucination, life threatening
58
Cutaneous porphyrias
affect skin, it becomes fragile and blistered leading to infections, and hair apigment changes and hair growth
59
Erythropoietic , bone marrow porphyrias
low # RBCs, spleen enlargement
60
Hepatic porphyrias
abnormal liver function, increased risk of liver cancer
61
Aquired porphyrias
no genetic, drug induced or Lead poisoning
62
What dose Lead poisonong do?
Inhibits Bone marrow enzyme key to heme synth, then reactive radical heme precurser builds up that can cause liver cancer
63
What do RBCs of someone with lead posioning look like
basophilic stippling
64
How are porphyrias lab tested?
blood, random or 24 hour blood or stool
65
when should sample be taken?
during acute attack
66
what does lab testing for porphyrias test
porphorins and toxic precursors that have built up.
67
what does decreased RBC lead to?
decreased hemoglobin and hematocrit
68
most common method to measure total hmopglobin content?
spectrophotometric analysis
69
what may interfere with hemoglobin total count?
High WBC, lipidemia, RBC abnormalitis, increased turbidity or increased bilirubin
70
what may increase hemoglobin
more RBC, COPD, Pulmonary fibrosis, HD, decreased blood volume and polycythemia vera, renal tumors
71
what may decrease hemogloben levels
less RBC, hemoglobinopathies, lead poisonongs, iron def, increase blood bolume
72
Hypochromasia
central pallor more than 1/3 of diamieter
73
where is hypochromasia found
iron deff, thalassemia
74
hematocrit
volume of erythrocyts as a percentage of volume of whole blood
75
what is the rule of three
hematocrit is aprox 3 times the hemoglobon valume whent RBCs are normal in every way
76
how is hematocrit now calculated?
computers based on RBC cound and size
77
normal hematocrit
42
78
what increases hematocrit
COPD, dehydration, polycythemia vera, congenital heart dz and erythrocytosis
79
what decreases hematocrit
anemia, hemoglopinopathise, cirrhosis,
80
what can effect hematocrit?
Larger RBC, high WBC count, hydration, pregnancy, altitudes, post hemorrhage, drugs
81
what is the RBC indices?
describe size and hemoglobin content of RBC, (hematocrit, hemoglobin level, and RBC size)
82
what are the RBC indices used for?
Dxx of anemia, by providing info on size, weight and concentration
83
MCV
size - mean corpuscular volume of RBC indices
84
MCH
hemoglobin weight
85
MCHC
hemoglobin concentration of RBC, weight to volume ratio of concentration of Hgb in RBC
86
what is mean corpuscular volume
average volume of RBC
87
normal MCV
82-97 fl
88
RBC histogram
relationship between RBC size and number
89
How is anemia classified
microcytic, normocytic or microcytic based on MCV
90
what can mess up MCV
high wbc, large platelets, agglutinate rbc fragment
91
what does MCH follow?
MCV, bigger cells have more Hgb, smaller have less
92
what is MCHC used for?
to classify anemia , normochromic, hypochromic
93
what about hypochromic MCHC
cant happen, the limit is 37 that?s as many as can fit/ratio.
94
what may machines call hyperchromic MCHC
sherocytosis, hyperlipidemia, cold agglutinins, rouleaus formation
95
so MCHC above 37 meanse
sherocytosis, hyperlipidemai or some other RBC problem that lowers machine RBC count
96
normocytic, normochromic anemias
chronic illnes, renal dz, blood loss, aplastic anemia, acquired hemolytic anemai
97
microcytic hypochromic
Fe difficiency, sideroblastic, lead poisoning, anemia of later stage chronic dz, thalassemia
98
Macrocytic normochromic
B12, folic acid def, pernicious anemia, chemotherapy, myelodysplastic syndrom, precurser to acute myleogenous leukemia