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Lab diagnosis > Week 5 lab diagnosis lecture > Flashcards

Week 5 lab diagnosis lecture Flashcards

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
Q

symptoms of major thalassemia

A

anemia in first year, jaundice, facial bone deformities, growth failure and shortness of breath

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26
Q

what would blood from Beta thalassemia major look like?

A

poikilocytic RBC, so lots of different shapes

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27
Q

what is the most common form of beta thalassemia

A

Beta thalassemia minor where only one beta chain is affected

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28
Q

Beta thalassema minor looks like what?

A

mild microcytic hypochromic anemia with no other clinical symptioms., so RCBs are smal with less color

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29
Q

what is hemoglobin S (sickel) ?

A

Predominant in those with sickel sell, variation on beta chain

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30
Q

Hemoglobin C?

A

disease with mild hemolytic anemia and splenomegaly

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31
Q

Hemoglobin E?

A

Mild hemolytic anema and splenomegaly, very common in SE asia

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32
Q

Hemoglobin constan spring

A

alpha chain is abnormally long resulting in thalassemic phenotype.

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33
Q

Hemoglobin H?

A

made of 4 beta chains because Alphas arnt made

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34
Q

Hemoglobin barts

A

No alpha chain produced, die in utero moslty

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35
Q

Hemoglobinopathies

A

inherited defects in globin structure mostly involving a single aa substitution

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36
Q

what makes sickle cells change shape

A

Glutamic acid instead of valine allows hemoglobin to polymerize, link inot long stiff structure that stretches out RBC

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37
Q

How long does sickel cell last?

A

20 or less days, its also rigid

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38
Q

Baso-occlusion

A

sickle cells block blood to microscopic regions of tissue because of their shape and regidity, this causes hypoxia of tissued, pain and organ damage

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39
Q

what % has sickel cell trait, but are heterozygous

A

8 % of african americans, without evidence of dz mostly, may have splenic infarcts if hypoxia or hematuria

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40
Q

For heterozygous sickel what is the % of Hgb S?

A

20-40% of hemoglobin is Hgb S

41
Q

For homozygous sickle cell, what % of S hemoglobin

A

80-100 %

42
Q

what % has sickle cell disease

A

less than 1 % of african americans

43
Q

when does sickle cell disease show?

A

after 6 months, then show anema with slight jaundice

44
Q

symptoms of sickel cell?

A

die before 40, infarcts in various organs and bone pain

45
Q

What is hemoglobin D disease

A

similar to HbS but lysine is substatuted in, causing milded hemolytic andmia

46
Q

what happens to RBCs in hemoglobin D instead of shape change

A

precipitation of hemoglobin crytalize, so they get removed by spleen, reducing risk for malaria

47
Q

Hemoglobin C dz. Who has it?

A

3% of African american from Africa

48
Q

what does Hb C dz do?

A

reduction of RBC lifespan, abdominal and bone pain, large number of target cells, less severe thatn sickle dz

49
Q

why may HbS reduce malaria?

A

reduce oxygen tension in cells retarding parasite growth

50
Q

why may A thalassema reduce malaria?

A

if gets mild malaria is now immune to more severe form

51
Q

Why B thalassema reduce malaria?

A

?, maybe reduction in cell adhesion in cerebral form of malaria

52
Q

where does the heme synthesis happen

A

85% in RBC but it quits when they mature

53
Q

where does the rest of the heme synthesis happen

A

mostly the liver, its used for synthesis of cytochrome p450 enzymes for detox

54
Q

what are Porphorias?

A

dz that are caused by deficiencies of enzymes of the heme biosynthetic pathy

55
Q

what are the two main porphorias?

A

Acute intermetted porhyria, porphyria cutanea darda

56
Q

what are the two cardinal symptoms of those with porphorya

A

Photosensitivity and neurologic disorders

57
Q

Acute porphyrias

A

affect NS, quickly start and end, abdominal pain, vomiting, diarrhea, constipation, hallucination, life threatening

58
Q

Cutaneous porphyrias

A

affect skin, it becomes fragile and blistered leading to infections, and hair apigment changes and hair growth

59
Q

Erythropoietic , bone marrow porphyrias

A

low # RBCs, spleen enlargement

60
Q

Hepatic porphyrias

A

abnormal liver function, increased risk of liver cancer

61
Q

Aquired porphyrias

A

no genetic, drug induced or Lead poisoning

62
Q

What dose Lead poisonong do?

A

Inhibits Bone marrow enzyme key to heme synth, then reactive radical heme precurser builds up that can cause liver cancer

63
Q

What do RBCs of someone with lead posioning look like

A

basophilic stippling

64
Q

How are porphyrias lab tested?

A

blood, random or 24 hour blood or stool

65
Q

when should sample be taken?

A

during acute attack

66
Q

what does lab testing for porphyrias test

A

porphorins and toxic precursors that have built up.

67
Q

what does decreased RBC lead to?

A

decreased hemoglobin and hematocrit

68
Q

most common method to measure total hmopglobin content?

A

spectrophotometric analysis

69
Q

what may interfere with hemoglobin total count?

A

High WBC, lipidemia, RBC abnormalitis, increased turbidity or increased bilirubin

70
Q

what may increase hemoglobin

A

more RBC, COPD, Pulmonary fibrosis, HD, decreased blood volume and polycythemia vera, renal tumors

71
Q

what may decrease hemogloben levels

A

less RBC, hemoglobinopathies, lead poisonongs, iron def, increase blood bolume

72
Q

Hypochromasia

A

central pallor more than 1/3 of diamieter

73
Q

where is hypochromasia found

A

iron deff, thalassemia

74
Q

hematocrit

A

volume of erythrocyts as a percentage of volume of whole blood

75
Q

what is the rule of three

A

hematocrit is aprox 3 times the hemoglobon valume whent RBCs are normal in every way

76
Q

how is hematocrit now calculated?

A

computers based on RBC cound and size

77
Q

normal hematocrit

A

42

78
Q

what increases hematocrit

A

COPD, dehydration, polycythemia vera, congenital heart dz and erythrocytosis

79
Q

what decreases hematocrit

A

anemia, hemoglopinopathise, cirrhosis,

80
Q

what can effect hematocrit?

A

Larger RBC, high WBC count, hydration, pregnancy, altitudes, post hemorrhage, drugs

81
Q

what is the RBC indices?

A

describe size and hemoglobin content of RBC, (hematocrit, hemoglobin level, and RBC size)

82
Q

what are the RBC indices used for?

A

Dxx of anemia, by providing info on size, weight and concentration

83
Q

MCV

A

size - mean corpuscular volume of RBC indices

84
Q

MCH

A

hemoglobin weight

85
Q

MCHC

A

hemoglobin concentration of RBC, weight to volume ratio of concentration of Hgb in RBC

86
Q

what is mean corpuscular volume

A

average volume of RBC

87
Q

normal MCV

A

82-97 fl

88
Q

RBC histogram

A

relationship between RBC size and number

89
Q

How is anemia classified

A

microcytic, normocytic or microcytic based on MCV

90
Q

what can mess up MCV

A

high wbc, large platelets, agglutinate rbc fragment

91
Q

what does MCH follow?

A

MCV, bigger cells have more Hgb, smaller have less

92
Q

what is MCHC used for?

A

to classify anemia , normochromic, hypochromic

93
Q

what about hypochromic MCHC

A

cant happen, the limit is 37 that?s as many as can fit/ratio.

94
Q

what may machines call hyperchromic MCHC

A

sherocytosis, hyperlipidemia, cold agglutinins, rouleaus formation

95
Q

so MCHC above 37 meanse

A

sherocytosis, hyperlipidemai or some other RBC problem that lowers machine RBC count

96
Q

normocytic, normochromic anemias

A

chronic illnes, renal dz, blood loss, aplastic anemia, acquired hemolytic anemai

97
Q

microcytic hypochromic

A

Fe difficiency, sideroblastic, lead poisoning, anemia of later stage chronic dz, thalassemia

98
Q

Macrocytic normochromic

A

B12, folic acid def, pernicious anemia, chemotherapy, myelodysplastic syndrom, precurser to acute myleogenous leukemia

Lab diagnosis (4 decks)

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