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Hemo 2 > Test 2 Continued > Flashcards

Test 2 Continued Flashcards

1
Q

A normal hemoglobin is composed of what?

A

4 hemo molecules, and 4 globin polypeptide chains

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2
Q

What are the normal adult hemoglobin’s?

A

Hgb a Hgb A2 and Hgb F

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3
Q

What does hemoglobin a consist of?

A

Two alpha and two beta chains it makes up 95 to 98% of hemoglobin

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4
Q

What does hemoglobin A2 consist of?

A

Two alpha and two Delta chains it makes up 2 to 3% of hemoglobin

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5
Q

What does hemoglobin F consist of?

A

Two alpha and two gamma chains it makes up less than 1% of hemoglobin

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6
Q

How are hemoglobinopathies acquired?

A

They are inherited

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7
Q

What genetic mutation occurs in hemoglobinopathies?

A

Amino acid sequence in the globe and chain is altered

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8
Q

What are the four abnormal functions/alterations that cause hemoglobinopathies

A

Single amino acid substitution in one of the chains, abnormal synthesis of one of the amino acid chains, fusion of hemoglobin chains, extension of an amino acid

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9
Q

What chain is normally altered in single amino acid substitution?

A

Beta

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10
Q

How Does a single amino acid substitution in one of the chains change the function?

A

Little to no effect on the globin chain function

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11
Q

What causes hemoglobin S?

A

Valine is substituted for glutamic acid at the six position of the beta chain

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12
Q

What causes hemoglobin C?

A

Lysine is substituted for glutamic acid at the sixth-position of the beta chain

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13
Q

What does abnormal synthesis of one of the amino acid chains result in?

A

It reduces or completely preclude the synthesis of one or more of the globe in chains (thalassemia)

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14
Q

What does fusion of hemoglobin chains result in?

A

Two normal genes break, then switch places and fuse together

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15
Q

What is the result of an extension of an amino acid chain?

A

The globin chains are longer than normal

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16
Q

If someone has hemoglobin disease, which alleles would have been inherited?

A

Either a homozygous for abnormal jeans or heterozygous for dominant gene that will cause an abnormality

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17
Q

What is an example of a hemoglobin disease?

A

Sickle cell anemia disease

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18
Q

In hemoglobin trait what alleles would be present?

A

Heterozygous As

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19
Q

What is an example of a hemoglobin trait?

A

Sickle cell trait 75% HGBA, 23% HGB S, 2% HGB A2

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20
Q

What is the main way to evaluate hemoglobinopathies?

A

Hemoglobin electrophoresis

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21
Q

Is hemoglobin electrophoresis a quantitative or qualitative test?

A

Quantitative

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22
Q

What does hemoglobin electrophoresis identify?

A

The types of hemoglobin based on electrophoretic mobility

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23
Q

What are three things that the migration of hemoglobins is dependent on in hemoglobin electrophoresis?

A

PH, time of migration, media used whether it be alkaline or acidic

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24
Q

What are some other ways to evaluate hemoglobinopathies?

A

DNA based gene analysis, Hgb F flow cytometry, HPLC

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25
Q

What is the proper way to use international nomenclature when naming variant hemoglobin?

A 
1 mutated chain identified
2 amino acid position affected
3 helical position of mutation 
4 amino acid substitution
Hgb S B6 Glu->Val
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26
Q

Hemoglobin F has a major hemoglobin component at what time in life?

A

Fetus and newborn

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27
Q

By what age have most people converted the hemoglobin F to hemoglobin a?

A

Two

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28
Q

What happens to hemoglobin F if it doesn’t convert to hemoglobin a as a patient ages?

A

It isn’t necessarily harm for however hemoglobin F doesn’t release oxygen as well as hemoglobin a

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29
Q

What is the lifespan of a red blood cell with hemoglobin S?

A

10 to 20 days

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30
Q

What hemoglobin lessens the severity of sickle cell anemia?

A

Hemoglobin F

31
Q

What are two primary test for hemoglobin S?

A

Newborn screening and solubility testing

32
Q

In solubility testing does a turbid solution represent Hgb S being present or not present?

A

HGBS present

33
Q

What does the amount of in vivo sickling depend on?

A

Hgb S

34
Q

If an individual is homozygous for hemoglobin S what are they?

A

Hemoglobin S anemic

35
Q

A crisis brings a patient in what are possible causes of a crisis?

A

Hypoxia, acidosis, dehydration, fever, cold stress And infections

36
Q

Children are usually symptom-free of hemoglobin S anemia because?

A

Because of the production of HGBF that ends around six months

37
Q

What are the laboratory results of sickle cell disease?

A

Hgb decreased between 5 to 9.5, hematocrit decreased 20 to 25%, red blood cells decreased reticulocytes increased white blood cells increased Bilirubin and lactate dehydrogenase increased

38
Q

What does a peripheral smear of someone with sickle cell disease show?

A

Normocytic/normochromic poikilocytosis sickle cells target cells and spherocytes
Anisosytosis. Polychromasia nucleated red blood cells Howell Jolie bodies basophilic stippling and pepper Heimir bodies
80% Hgb S 5% Hgb A2 Hgb F 1-20%

39
Q

What are treatments for sickle cell disease?

A

Red blood cell transfusion’s, hydroxyurea, prophylactic antibiotics

40
Q

In sickle cell trait the individual is heterozygous with what alleles?

A

Hgb A and S

41
Q

What are the laboratory results in sickle cell trait?

A

So you ability test positive, normal peripheral smear, 60% hemoglobin a and 40% hemoglobin S

42
Q

What happens with hemoglobin C?

A

Crystals form and give the red blood cells are rectangular shape

43
Q

What are the laboratory results in hemoglobin C disease?

A

Hemoglobin 9 to 12 red blood cell lifespan is 38 days peripheral smear normal city/normochromic with target cells, Spherocytes, small folded cells due to hemoglobin see crystals

44
Q

What do you need to know about hemoglobin c trait?

A

60% hemoglobin a 40% hemoglobin C with normal peripheral blood smear

45
Q

What are the laboratory results for hemoglobin SC disease?

A

Mild to moderate anemia hemoglobin 8 to 10, red blood cells life span of 29 days, peripheral smear will show target cells and possible folded cells

46
Q

What is thalassemia?

A

Decreased production of alpha or beta globin chains leading to an excess of other chains

47
Q

How are thalassemias acquired?

A

They are inherited

48
Q

What determines the type of thalassemia an individual has?

A

The globin chain affected

49
Q

In Alpha Thalassemia what synthesis is impaired and what access chains are present?

A

Alpha chain is impaired Causing an excess in beta chains

50
Q

What is the inherited genes in someone with thalassemia minor?

A

Heterozygous

51
Q

People with thalassemia major have inherited how many thalassemia genes?

A

Two homozygous

52
Q

When do thalassemias appear in individuals?

A

After the hemoglobin F synthesis stops at about one year

53
Q

What are symptoms of thalassemia?

A

In large spleen, liver, and heart thin and brittle bones due to ineffective erythropoiesis

54
Q

What is the leading cause of death in patients with thalassemias?

A

Heart failure and infections

55
Q

If someone with thalassemia is treated what might occur?

A

Iron deposits

56
Q

What is used to treat the iron deposits?

A

Chelating drugs

57
Q

What are two kinds of alpha thalassemia’s?

A

Accumulation of gamma tetramers (Barts hemoglobin) or accumulation of beta tetramers (hemoglobin H)

58
Q

If someone has only two viable alpha jeans meeting there were two deletions what do they have?

A

Alpha thalassemia trait

59
Q

What are the laboratory results of someone with alpha thalassemia trait?

A

Peripheral smear will show microcytic, hypochromic cells target cells and elliptocytes

60
Q

How many functioning alpha genes does someone with hemoglobin H disease have?

A

1

61
Q

What are the laboratory findings of someone with HGBH?

A

Microcytic, hypochromic cells, target cells, poikilocytosis, Anisocytosis, schistocytes, basophilic stippling, Hgb H inclusions

62
Q

What is hydrops fetalis?

A

The most severe state of Alpha thalassemia with no functioning Alpha genes
No HGBA only Hgb Barts

63
Q

Hgb Bart has high oxygen affinity resulting in what?

A

Resistance of delivering oxygen to tissues causing fetus to die of hypoxia

64
Q

Laboratory findings of hydrops fetalis?

A

Marked microcytic, hypochromic anemia, marked anisocytosis, marked poikilocytosis, numerous nucleated red blood cells

65
Q

Beta thalassemia’s results in what?

A

Extravascular homolysis

66
Q

Beta thalassemia minor also known as coolies trait laboratory results would include?

A

Decrease osmotic fragility, microcytic hypochromic cells, basophilic stippling, target cells, elliptocytes, hemoglobin electrophoresis would show an increase in Hgb A2

67
Q

Betos Alcenia major/Cooleys anemia has a little or no what synthesized?

A

Hemoglobin a

68
Q

What are the laboratory findings of someone with thalassemia major?

A

Severe anemia red blood cell life span of 7 to 22 days Hgb between two and nine, decreased red blood cells MCV osmotic fragility increased white blood cells reticulocytes Billy Rubin total iron binding capacity in serum iron
Peripheral smear would show hypochromic microcytic cells with Marc poikilocytosis literally everything

69
Q

What are treatments for thalassemia major?

A

Transfusions, bone marrow and stem cell transplant’s, drugs like hydroxyurea, gene therapy

70
Q

HGBSB to thalassemia has what percentage of HGBS present?

A

65 to 95%

71
Q

What are the lab findings of someone with HGBS beta thalassemia?

A

Hgb of 8 to 9 with increased reticulocytes peripheral smear will show microcytic hypochromic cells with nucleated red blood cells target cells polychromasia sickle cells and Ani so cytosis Hgb electrophoresis will show three bands present one at HTBS, HGBA2, Hgb F

72
Q

What differentiates beta thalassemia’s from sickle cell trait?

A

In beta thalassemia as you’ll see increased Hgb A2in microcytes in sickle cell you’ll see HGBA is the predominant type

73
Q

What should you look for to differentiate thalassemias from iron deficiency’s?

A

Thalassemias: RBC increased, basophilic stippling, target cells, serum iron increased, complete transferrin saturation, osmotic fragility decreased
Iron deficiency: RBC decreased, basophilic stippling will only appear after treatment, occasional target cells, osmotic fragility is normal

Hemo 2 (7 decks)

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