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Hematology Module 1 and 2 > Normocytic-Normochromic Anemias > Flashcards

Normocytic-Normochromic Anemias Flashcards

1
Q

What is the most common cause of aplastic anemia?

A

Idiopathic (unknown)

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2
Q

What are the four secondary causes for aplastic anemia?

A

Chemicals, Drugs (chloramphenical), Radiation, and infections (Chronic)

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3
Q

What is the most common congenital disorder associated with aplastic anemia?

A

Fanconi’s anemia

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4
Q

What is the BM cellularity of aplastic anemia?

A

Hypocellular

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5
Q

What is the CBC results of someone with aplastic anemia?

A

Pancytopenia Decreased in HGB and HCT, Normocytic normochromic.

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6
Q

What is the RBC morphology of someone with aplastic anemia?

A

No characteristic; few present.

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7
Q

What is the retic count for someone with aplastic anemia?

A

Decreased to absent.

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8
Q

What is the treatment for someone with aplastic anemia?

A

Take away offending agent, support therapy, immunosuppressive therapy. Cure = BM transplant

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9
Q

What type of Poik is in every hemoglobinapathy?

A

Targets

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10
Q

What is the amino acid substitution for someone with sickle cell?

A

Valine for glutamic acid

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11
Q

What are the 3 factors contributing to sickling process?

A

Hypoxia, acidosis (CO2) and Dehydration

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12
Q

What is the cause for painful crisis in SC?

A

Infection, fever, dehydration, exposure to extreme cold (tissue damage)

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13
Q

What are the causes for acute chest syndrome in SC?

A

Infarction of lungs

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14
Q

What are the causes for high risk of infection in SC?

A

Tissue damage in spleen

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15
Q

What is the difference from SC disease and SC trait in regards to inheritance?

A

SCD: inherited two sickle genes, one from each parent.
SCT: Inherited one normal gene from one parent and one sickle gene from the other

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16
Q

What is the difference from SC disease and SC trait in regards to hemoglobin nomenclature?

A 
AS = Trait
SS = Disease
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17
Q

What is the difference from SC disease and SC trait in regards to solubility results?

A

AS and SS remain turbid and are insoluble. only AA is soluble and is thus clear

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18
Q

What is the difference from SC disease and SC trait in regards to hemoglobin electrophoresis results?

A

AS: two bands A>S
SS: S>F (no A)

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19
Q

What is the difference from SC disease and SC trait in regards to RBC morphology?

A

SS: Targets, sickles, schistocytes, sphereocytes
AS: Slight targets, usually no sickles unless in crisis

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20
Q

What is the difference from SC disease and SC trait in regards to treatment?

A

SS: Hydration, Morphine, antibiotics, blood transfusions
AS: No treatment

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21
Q

What is the principle of the sickledex solubility test?

A

Reduction; S- HGB is insoluble, A is soluble

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22
Q

What is the reagent for the sickledex solubility test?

A

Sodium dithionite or sodium metabisulfite

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23
Q

What are the causes for false positives in the sickledex solubility test?

A

Protienemia, >18g/dL, other sickling HGBs

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24
Q

What are the causes for false negatives in the sickledex solubility test?

A

Newborn, HGB

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25
Q

What is the amino acid substitution for someone with hemoglobin C disease?

A

Lysine for glutamic acid

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26
Q

What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to clinical presentation?

A

CC: mildly hemolytic; splenomegaly
AC: Asymptomatic

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27
Q

What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to hemoglobin nomenclature?

A 
CC = disease
AC = trait
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28
Q

What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to hemoglobin electrophoresis?

A 
CC = 100% C, (no A)
AC = A>C
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29
Q

What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to RBC morphology?

A

CC: Targets +, Crystals, polychromes, (increase in retic)

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30
Q

What is the inheritance for SC disease?

A

Lysine sub from one parent, Valine sub from the other

31
Q

What is the clinical presentation for SC disease?

A

Less sever than sickle but severe hemolytic anemia with painful crisis

32
Q

What is the electrophoresis result for someone with SC disease?

33
Q

What is the RBC morphology for someone with SC disease?

A

Tartget +, C-crystals, S-C Crystals, polychromasia, H-J, Pappenheimer, nRBC

34
Q

Review worksheet 7 for electrophoretic patter in cord blood, CC, SC, AC, AS, and SS

35
Q

What is the inheritance for someone with sickle cell-Beta thal?

A

Valine suc from one and B^o or B^+ from the other

36
Q

What are the clinical presentations for someone with sickle cell-Beta thal major vs. minor?

A

Major: severe hemolytic anemia
Minor: Mild to moderate

37
Q

What are the hemoglobin electrophoresis results for someone with sickle cell-Beta thal major vs. minor?

A

Major: S>F (^)> A2 (^) (no A)
Minor: S>A>F (^)>A2

38
Q

What is the RBC morphology for someone with sickle cell-Beta thal major vs. minor?

A

Targets +, sickles, nRBC, hemolytic poik

Sickles may be absent in minor

39
Q

What are the two ways that Hemoglobin D disease is differentiated from Hemoglobin S?

A

Solubility and Citrate electrophoresis

40
Q

What is the worlds third most common abnormal hemoglobin and where does it occur?

A

Hemoglobin E disease, SE Asia`

41
Q

What is the mechanism for hereditary sphereocytosis?

A

Decrease in spectrin = Increase permeability of Na+ into cell

42
Q

What is the mechanism for hereditary elliptocytosis?

A

Decrease in cholesterol causing HGB to polarize to opposite sides of cell

43
Q

What is the mechanism for hereditary stomatocytosis?

A

Defect in Na+/K+ pump

44
Q

What is the clinical presentation for hereditary sphereocytosis?

A

Anemia, jaundice and splenomegaly

45
Q

What is the RBC indices for hereditary sphereocytosis?

A

HGB ~ 12g/dL

MCHC = 36-38%

46
Q

What is the RBC morphology for hereditary sphereocytosis?

A

Variable number of spheres, polychromasia, increased retic

47
Q

What is the principle for the osmotic fragility test?

A

Blood to series of hypotonic salt, hemolysis observed

48
Q

Conditions that show increased osmotic fragility?

A

Sphereocytosis

49
Q

Conditions that show decreased osmotic fragility?

A

Thals, sickles, hypochromia

50
Q

Conditions that show increased resistance to hemolysis?

A

Thals, sickles, hypochromia

51
Q

Conditions that show decreased resistance to hemolysis?

A

Sphereocytosis

52
Q

What is the NaCl [ ] when hemolysis should begin?

A

0.45-.50% NaCl

53
Q

What is the NaCl [ ] when hemolysis should be completed?

A

0.3-0.35% NaCl

54
Q

What type of pork demonstrate the greater resistance to hemolysis?

A

Targets (hypochromia and sickles)

55
Q

In someone with any hemolytic anemia, what are the expected results for plasma hemoglobin?

56
Q

In someone with any hemolytic anemia, what are the expected results for retics?

57
Q

In someone with any hemolytic anemia, what are the expected results for serum bilirubin?

58
Q

What is the deficient enzyme in G-6-PD?

A

Reduced Glutathione

59
Q

What is the deficient enzyme in PK deficiency?

A

Pyruvate Kinase, = Burr

60
Q

What is the deficient enzyme in methemoglobin reductase?

A

Methemoglobin (ferric) Cyanotic

61
Q

What is the triggering factors in G-6-PD?

A

New drug, Infection, Feva beans, ingestion of moth balls

62
Q

What is the etiology of PNH?

A

RBCs are more sensitive than normal to lytic action of complement.

63
Q

What is the Clinical presentation of PNH?

A

Sleep induced hemolytic action, bloody first urine

64
Q

What is the CBC for PNH?

A

Panocytopenia

65
Q

What is the RBC morphology for PNH?

66
Q

What test is done to confirm PNH and what is its result?

A

Positive HAMS test

67
Q

What is the principles of Hams test?

A

Complement to alternative pathway = lysis of PNH cells

68
Q

What are the three conditions that may cause illumine hemolytic anemia?

A

Transfusions, Pregnancy and organ transplant

69
Q

What are the differences between autoimmune and alloimmune hemolytic anemia?

A 
Auto = self
Allo = antibodies one one react with antigens of another
70
Q

What is the etiology of cold agglutination?

A

??????? Clumping of RBC cut to IgM antibody

71
Q

What is the CBC results of Cold agglutination?

A

Break rule of 3 = high HCV

72
Q

What antibody is associated with Paroxysmal cold hemoglobinuria?

A

Donath-lansteiner antibody

73
Q

What are the two disorders associated with MAHA?

A

hemolytic uremic syndrome and Thrombotic thrombocytopenic purpura

74
Q

What is the predominate poik in MAHA?

A

Schistocytes

Hematology Module 1 and 2 (9 decks)

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