Normocytic-Normochromic Anemias Flashcards Preview

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

Flashcards in Normocytic-Normochromic Anemias Deck (74):
1

What is the most common cause of aplastic anemia?

Idiopathic (unknown)

2

What are the four secondary causes for aplastic anemia?

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

3

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

Fanconi's anemia

4

What is the BM cellularity of aplastic anemia?

Hypocellular

5

What is the CBC results of someone with aplastic anemia?

Pancytopenia Decreased in HGB and HCT, Normocytic normochromic.

6

What is the RBC morphology of someone with aplastic anemia?

No characteristic; few present.

7

What is the retic count for someone with aplastic anemia?

Decreased to absent.

8

What is the treatment for someone with aplastic anemia?

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

9

What type of Poik is in every hemoglobinapathy?

Targets

10

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

Valine for glutamic acid

11

What are the 3 factors contributing to sickling process?

Hypoxia, acidosis (CO2) and Dehydration

12

What is the cause for painful crisis in SC?

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

13

What are the causes for acute chest syndrome in SC?

Infarction of lungs

14

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

Tissue damage in spleen

15

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

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

16

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

AS = Trait
SS = Disease

17

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

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

18

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

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

19

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

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

20

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

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

21

What is the principle of the sickledex solubility test?

Reduction; S- HGB is insoluble, A is soluble

22

What is the reagent for the sickledex solubility test?

Sodium dithionite or sodium metabisulfite

23

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

Protienemia, >18g/dL, other sickling HGBs

24

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

Newborn, HGB

25

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

Lysine for glutamic acid

26

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

CC: mildly hemolytic; splenomegaly
AC: Asymptomatic

27

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

CC = disease
AC = trait

28

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

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

29

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

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

30

What is the inheritance for SC disease?

Lysine sub from one parent, Valine sub from the other

31

What is the clinical presentation for SC disease?

Less sever than sickle but severe hemolytic anemia with painful crisis

32

What is the electrophoresis result for someone with SC disease?

S = C

33

What is the RBC morphology for someone with SC disease?

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

34

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

Review!!

35

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

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

36

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

Major: severe hemolytic anemia
Minor: Mild to moderate

37

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

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

38

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

Targets +, sickles, nRBC, hemolytic poik
Sickles may be absent in minor

39

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

Solubility and Citrate electrophoresis

40

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

Hemoglobin E disease, SE Asia`

41

What is the mechanism for hereditary sphereocytosis?

Decrease in spectrin = Increase permeability of Na+ into cell

42

What is the mechanism for hereditary elliptocytosis?

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

43

What is the mechanism for hereditary stomatocytosis?

Defect in Na+/K+ pump

44

What is the clinical presentation for hereditary sphereocytosis?

Anemia, jaundice and splenomegaly

45

What is the RBC indices for hereditary sphereocytosis?

HGB ~ 12g/dL
MCHC = 36-38%

46

What is the RBC morphology for hereditary sphereocytosis?

Variable number of spheres, polychromasia, increased retic

47

What is the principle for the osmotic fragility test?

Blood to series of hypotonic salt, hemolysis observed

48

Conditions that show increased osmotic fragility?

Sphereocytosis

49

Conditions that show decreased osmotic fragility?

Thals, sickles, hypochromia

50

Conditions that show increased resistance to hemolysis?

Thals, sickles, hypochromia

51

Conditions that show decreased resistance to hemolysis?

Sphereocytosis

52

What is the NaCl [ ] when hemolysis should begin?

0.45-.50% NaCl

53

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

0.3-0.35% NaCl

54

What type of pork demonstrate the greater resistance to hemolysis?

Targets (hypochromia and sickles)

55

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

Decreased

56

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

Increased

57

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

Increased

58

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

Reduced Glutathione

59

What is the deficient enzyme in PK deficiency?

Pyruvate Kinase, = Burr

60

What is the deficient enzyme in methemoglobin reductase?

Methemoglobin (ferric) Cyanotic

61

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

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

62

What is the etiology of PNH?

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

63

What is the Clinical presentation of PNH?

Sleep induced hemolytic action, bloody first urine

64

What is the CBC for PNH?

Panocytopenia

65

What is the RBC morphology for PNH?

None

66

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

Positive HAMS test

67

What is the principles of Hams test?

Complement to alternative pathway = lysis of PNH cells

68

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

Transfusions, Pregnancy and organ transplant

69

What are the differences between autoimmune and alloimmune hemolytic anemia?

Auto = self
Allo = antibodies one one react with antigens of another

70

What is the etiology of cold agglutination?

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

71

What is the CBC results of Cold agglutination?

Break rule of 3 = high HCV

72

What antibody is associated with Paroxysmal cold hemoglobinuria?

Donath-lansteiner antibody

73

What are the two disorders associated with MAHA?

hemolytic uremic syndrome and Thrombotic thrombocytopenic purpura

74

What is the predominate poik in MAHA?

Schistocytes