Hematology 2- B12, Folic Acid, Aplastic Anemia 10/21/16 Flashcards

1
Q

What type of cytic anemia with B12, Folic Acid, and Aplastic Anemia?

A

Macrocytic anemia

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2
Q

MCV over 100 and below 115 is…

A

Macrocytic

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3
Q

MCV over 115 is…

A

Megaloblastic

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4
Q

After determining PT has macrocytic anemia what to check next?

A

Reticulocyte count high or low

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5
Q

What does reticulocyte count check?

A

Gives idea as to bone marrow function. High is due to high output and pushing immature cells out=reticulocytosis

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6
Q

What is reticulocytosis?

A

Increase in reticulocytes (immature RBCs)

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7
Q

Normal Reticulocyte count range?

A

2.0-3.0

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8
Q

If Macrocytic and low Reticulocyte count then what to check? (Hint: 2 things)

A

Check if Hypersegmented Neutrophils and MCV above or below 115.

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9
Q

If Macrocytic, low Reticulocyte, Hypersegmented Neutrophils, and MCV above 115?

A

B12 or Folate deficiency

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10
Q

If Macrocytic, low Reticulocyte, no Hypersegmented Neutrophils, and MCV below 115? (Hint: If not d/t meds or liver, thyroid, DS)

A

Probable myelodysplasia

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11
Q

What causes Macrocytic Anemia?

A

Abnormal DNA metabolism, shift to immature cells when anemic and pushing out immature cells too early, bone marrow disorder, lipid abnormalities, unknown dz

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12
Q

If Megaloblastic MCV above 115 two common causes?

A

B12 or Folate deficiency

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13
Q

If Macrocytic MCV above 100 common causes?

A

Reticulocytosis, alcoholism, liver dz, hypothyroid, meds, aplasic anemia

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14
Q

What stains blue in reticulocytosis?

A

RNA

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15
Q

B12 or Folate deficiency has MCV above what? High or low Reticulocyte count?

A

MCV over 115 (Megaloblastic). Low Reticulocyte.

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16
Q

Myelodysplasia MCV, Ret count, segmented?

A

MCV above 100 macrocytic, low ret count, not hypersegmented neutrophils.

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17
Q

90% of alcoholics have an MCV between what and what?

A

100-110

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18
Q

Alcoholic Macrocytic anemia resolves after how long of not drinking?

A

2-4 months

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19
Q

Alcoholics are commonly deficient in what even if taking supplements and will still be macrocytic?

A

Folic acid deficient

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20
Q

Macrocytic and hypothyroidism do to what?

A

Unknown. Check liver function, thyroid function, and vitamin deficiency.

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21
Q

What is the cause of medication-induced macrocytic anemia?

A

Combo antiretroviral therapy for HIV. Inhibition of various pathways. Hydroxyurea for sickel cell dz.

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22
Q

Myelodysplastic Syndromes due to issues of what? Causes inappropriately low response of what?

A

Issues of bone marrow itself. Inappropriately low reticulocyte response.

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23
Q

Aplastic Anemia is failure of what?

A

Bone marrow failure

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24
Q

What happens to all 3 cell lines in Aplastic Anemia?

A

Very low. Peripheral Pancytopenia.

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25
Q

Which 3 cell lines are low in Aplastic Anemia?

A

Platelets, RBCs, WBCs

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26
Q

Epi of Aplastic Anemia?

A

2-4 M/yr in US. 2-3x higher in East Asia. Men and women equally.

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27
Q

Distribution of Aplastic Anemia?

A

Biphasic. 10-25, over 60 years.

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28
Q

What are the two “phases” of injury to pluripotent stem cells in Aplastic Anemia?

A
  1. Damage to stem cells from viruses/drugs/chemo/radiation/toxins
  2. Autoimmune response against stem cells
    Both cause pancytopenia!
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29
Q

What is the major cause of autoimmune Aplastic Anemia?

A

Idiopathic

30
Q

Which two drugs are major causes of acquired Aplastic Anemia?

A

Chloramphenicol and Sulfonamides

31
Q

What is Fanconi Anemia?

A

Defect in DNA repair pathway. Cause congenital Aplastic Anemia.

32
Q

What is Dyskeratosis congenita?

A

Defect in maintenance of hematopoietic stem cell

telomere length maintenance. Cause congenital Aplastic Anemia.

33
Q

What are the 4 steps in Aplastic Anemia?

A
  1. Inciting event
  2. Aberrant immune response
  3. Destruction of hematopoietic stem cells
  4. Clinical disease
34
Q

After destruction of hematopoietic stem cells how long until pancytopenia seen? Which cells die first?

A

Weeks to month. WBCs die first, so infection seen first.

35
Q

Aplastic Anemia clinical presentation?

A
  • Weakness and fatigue
  • Cardiopulmonary compromise
  • Progressive anemia
  • Mucosal and skin bleeding
  • Infection
36
Q

What is common cause of death in Aplastic Anemia?

A

Invasive fungal infection

37
Q

Profound neutropenia causes…?

A

Recurrent infections

38
Q

Thrombocytipenia causes….?

A

Mucosal hemorrhage

39
Q

Aplastic Anemia causes what in premenopausal women?

A

Increased menstural flow

40
Q

Is PE for Aplastic Anemia helpful?

A

Not very

41
Q

What 2 most common PE findings in Aplastic Anemia?

A

Pallor, Pepechiae

42
Q

What 3 things should NOT be seen in Aplastic Anemia?

A
  1. Hepatosplenomegaly
  2. Lymphadenopathy
  3. Bone tenderness
43
Q

CBC shows what in Aplastic Anemia?

A

Pancytopenia. Anemia can be severe.

44
Q

Are abnormal cells present in Aplastic Anemia?

A

NO!

45
Q

Reticulocyte Count/Index in Aplastic Anemia?

A

Decreased

46
Q

Diagnostic criteria for Moderate Aplastic Anemia?

A
  • Bone marrow cellularity below 30%
  • Absence of severe cytopenia
  • Depression of at least two of three blood elements below normal
47
Q

Diagnostic criteria for Severe Aplastic Anemia (sAA)?

A
  • Bone marrow biopsy 25% below normal, OR

- BM bx 50% below norm with less than 30% hematopoietic AND very low reticulocyte, neutrophil, or platelet count

48
Q

Diagnostic criteria for Very Severe Aplastic Anemia (vsAA)?

A
  • sAA criteria met

- Absolute neutrophil count (ANC) below 200

49
Q

3 essentials for Aplastic Anemia dx?

A
  1. Pancytopenia
  2. No abnormal hematopoietic cells circulating or in BM
  3. Hypocellular bone marrow
50
Q

Tx for mild to moderate Aplastic Anemia?

A
  • Supportive
  • EPO or darbeopoetin
  • Myeloid growth factors
  • Transfusions
  • Abx or antifungals
51
Q

Tx for severe Aplastic Anemia?

A

Under 20=Bone marrow t/p

Under 50=Immunosuppressive therapy

52
Q

Aplastic Anemia IST suggested regimen?

A

Horse antithymocyte globulin(ATG) plus cyclosporin A (CsA)

53
Q

Untreated Aplastic Anemia 1 year mortality rate?

A

70% mortality rate

54
Q

Severe Aplastic Anemia (sAA) survival rates if correctly treated?

A

80-90%

55
Q

What is most common cause of congenital Aplastic Anemia?

A

Fanconi Anemia

56
Q

Eti of Fanconi Anemia?

A

Autosomal recessive disorder->defect in DNA repair pathway. Increased incidents of malignancies.

57
Q

Presentation of Fanconi Anemia

A

Congenital abnormalities: fused digits

58
Q

Fanconi Anemia tx?

A

Allogeneic hematopoietic cell transplant

59
Q

MCV of B12 and Folate deficiencies?

A

110-115. Megaloblastic.

60
Q

What is B12 involved in what can cause neurological issues?

A

Myelin production

61
Q

Megaloblastic anemia is when MCV is above what? What do RBCs look like?

A

MCV above 110. Macroovalocytic RBCs w/segmented neutrophils.

62
Q

Megaloblastic anemia and bone marrow?

A
  • Erythroid hyperplasia

- Megaloblastic morphology

63
Q

Most common causes of Megaloblastic anemia?

A
  • Faulty food intake/being a vegan

- Preggers

64
Q

Pernicious Anemia epi?

A

1:7500, over 40 y/o.

65
Q

How long are B12 stores?

A

3 years

66
Q

What produces Intrinsic Factor? Job?

A

Parietal cells in stomach. Absorbs B12 in terminal ileum.

67
Q

Where is B12 and IF absorbed?

A

Terminal ileum

68
Q

B12 cofactor for what 2 important functions?

A
  1. Myelin synthesis

2. Nucleic acid synthesis

69
Q

Pernicious Anemia eti?

A

Autoimmune. Antibodies destroy parietal cells, causing atrophic gastritis, and neutralizing IF.

70
Q

Pernicious Anemia causes higher risk for what?

A

Gi/gastric cancers. Endoscopy when diagnosed, monitor for occult blood.