Lecture 2b Flashcards

1
Q

Define aplastic anemia.

A

Failure of hematopoietic bone marrow due to suppression of injury to stem cells

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2
Q

What are the main causes of aplastic anemia?

A

Autoimmune supression
Benzene
Chemo
Hepatitis
Radiation exposure
Pregnancy

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3
Q

What are some symptoms of aplastic anemia?

A

Decreased WBC, RBC, and platelets
Leads to…
Infections
Anemia
Bruising, bleeding

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4
Q

Whats special about petechia and purpura?

A

They don’t blanch when pressure is put on it

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5
Q

Why are there no or very few reticulocytes in aplastic anemia?

A

The bone marrow isn’t functioning

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6
Q

What do you typically not see in aplastic anemia?

A

Hepatomegaly and spelnomegaly

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7
Q

What test do we use to check aplastic anemia?

A

Bone marrow biopsy

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8
Q

What are some treatments for aplastic anemia?

A

Remove underlying etiology
Red cell transfusion
Platelet transfusion
Multilineage: eltrombopag
Erthyropoietic: epoetin, darbepoetin
Myeloid: filgrastim, sargramostim
Bone marrow transplant
Triple therapy (immunosuppression, for those who can’t go through bone marrow transplant)

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9
Q

What is triple therapy?

A

Giving pts equine antithymocyte globulin(ATG), cyclosporine, and eltrombopag(promacta) for severe treatment of aplastic anemia
Steroids given along to reduce ATG side effects

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10
Q

How does Epoetin or Darbepoetin work?

A

EPO made with recombinate DNA that stimulates the erythroid precursors causing reticulocyte and RBC release
Rise in Hbg and Hct, dose-dependent (peaks 2-6weeks)

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11
Q

What is the different between Epoetin and Darbepoetin?

A

Darbepoetin has 3x the half life
Can be dosed weekly or Q 2 weeks

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12
Q

What are Epoetin and Darbepoetin indicated for?

A

Anemia from CKD
Chemotherapy
Myelodysplasia

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13
Q

What are the contraindications of Epoetin and Darbepoetin?

A

Allergy to drug
Uncontrolled HTN
Pure red cell aplasia after any EPO tx

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14
Q

What are some SE of Epoetin and Darbepoetin?

A

HTN
Thrombosis
Rash
Seizures
Pruritus
Fever
Edema
Dyspnea
Cough
Abdominal pain

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15
Q

What are the BBW for Epoetin and Darbepoetin?

A

Risk of death from thrombosis related issues
Increase Stoke in CKD with Hgb >11
Tumor progression of cancers
Increase risk of DVT after surgery

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16
Q

What do we need to monitor for patients on Epoetin and Darbepoetin?

A

Iron status
Hgb
BP

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17
Q

What is sideroblastic anemia?

A

Decrease of Hgb synthesis because of inability to make heme since it impairs the ability to incorporate iron into protoporhyrin IX(precursor to heme)

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18
Q

How is sideroblastic anemia inherited?

A

X-linked
Autosomal recessive
Mitochondrially inherited

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19
Q

How is sideroblastic anemia acquired?

A

Part of myelodysplastic syndrome
Alcoholism
Lead poisioning
Copper deficiency
Infection/inflammation
Medications: Isoniazid, Linezolid, Chloramphenicol

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20
Q

What other SE appears in sideroblastic anemia other than the normal anemic symptoms?

A

Palmar creases if Hgb <8-9 (also appears in iron deficient anemia)
May also see S/S with myelodysplastic syndrome

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21
Q

Why are reticulocytes decreased in sideroblastic anemia?

A

Pts have issue producing RBC

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22
Q

What shows up on a peripheral smear for sideroblastic anemia?

A

Basophilic stippling
Poikilocytosis
Anisocytosis
Polychromasia

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23
Q

What do we need to order before making a diagnosis of sideroblastic anemia?

A

Bone marrow aspirate

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24
Q

What does bone marrow aspirate show?

A

Erythroid hyperplasia: indicates effectiveness of erythropoiesis
Prussian blue stain: shows ringed sideroblasts and increase of iron stores

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25
Q

What are some treatments of sideroblastic anemia?

A

Transfusion
B6 (pyridoxine) or B1 (thiamine)
Stopping medication (isoniazid)
Genetic counseling
Phlebotomies, chelation (to prevent iron overload)

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26
Q

What is the main cause of anemia worldwide?

A

Iron deficient anemia

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27
Q

How much iron do we need to intake a day?

A

10-15mg

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28
Q

How much iron do we absorb?

A

10%

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29
Q

What is the role of ferroportin?

A

Iron transporter that release iron from cells

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30
Q

What dos Hepcidin do to ferroportin?

A

Promotes ferroportin breakdown which inhibits iron release

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31
Q

How much iron do we lose a day?

A

Lose 1 mg from skin/mucosa
So we absorb ~1mg and lose 1mg

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32
Q

What are some causes of iron deficiency anemia?

A

Deficient diet
Increased iron requirements
Chronic blood loss
Decreased absorption
Iron sequestration

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33
Q

When do we need an increase of iron consumption?

A

Pregnancy
Lactation
Growth spurt

34
Q

What can cause a decreased absorption of iron?

A

Gastritis
Chronic disease
Gastric surgery
Zinc deficiency
Hereditary iron-deficency anemia

35
Q

What are the S/S of iron deficient anemia?

A

Normal anemic symptoms
Smooth tongue
Brittle nails
Koilonychia
Cheilosis
Plummer-Vinson syndrome
Restless leg
Neurodevelopmental delay

36
Q

What is Pica?

A

Craving for substances not rich in iron (ice, clay, dirt)

37
Q

What are the stages of iron deficiency?

A

Low iron without anemia
Normocytic anemia
Microcytic anemia

38
Q

What shows up on a peripheral smear in iron deficiency anemia?

A

Hypochromic microcytic cells
Target cells
Poikilocytosis
Anisocytosis
Increased platelets

39
Q

What are some treatments for iron deficiency anemia?

A

Supplements (Ferrous sulfate)
Transfusion
Iron replacement therapy (parenteral, IV)

40
Q

What can you add to increase absorption of ferrous sulfate?

A

Vitamin C

41
Q

What are the SE and dosage of ferrous sulfate?

A

325mg 3x/day on empty stomach
SE:
N/V
Constipation
Abdominal pain
Dark stool
Rare SE:
Diarrhea
Urine discoloration
Teeth staining

42
Q

What is contraindicated for ferrous sulfate?

A

Allergy to drug
Hemochromatosis
Hemolytic anemia

43
Q

What improved over iron dextran(older parenteral iron)?

A

New preparations are infused over minutes, not hours
Lower risk of serious reactions(anaphylaxis)
No risk of “iron staining” (cutaneous siderosis)

44
Q

What causes anemia of inflammation/infection?

A

Cytokines increased hepcidin which decrease iron absorption and availability
Mimics iron deficiency anemia
75% normocytic 25% microcytic

45
Q

Define anemia of CKD.

A

Anemia due to inadequate secretion of EPO by kidneys
Normocytic, normochromic anemia
Normal iron
Pts on dialysis can develop secondary iron/folate deficiency

46
Q

Define anemia of endocrine disorders.

A

Decrease EPO secretion
Normocytic, normochromic anemia
Decreased thyroid, testosterone, cortisol

47
Q

Define anemia of chronic liver disease.

A

Cholesterol deposits in RBC membrane causing decreased RBC survival and EPO sectretion
Macrocytic anemia due to deposits

48
Q

Define Anemia of starvation.

A

Decreased EPO due to decrease of metabolism(protein intake)

49
Q

Why are elderly susceptible to anemia?

A

Resistance to EPO
Decreased EPO secretion
Chronic low-level inflammation

50
Q

What are some treatments for anemia of chronic diseases?

A

Transfusion
EPO (Poetin, darbepoetin)

51
Q

What is the role of vitamin B12(cobalamin)?

A

Converts methylmalonyl-CoA to succinyl-CoA
Converts homocysteine to methionine
DNA synthesis in erythroid precursors

52
Q

What are sources of vitamin B12?

A

Animal-based foods, fortified foods

53
Q

How much B12 do we absorption and use?

A

Absorb ~5mcg/day
Use 3-5 mcg/day

54
Q

How much B12 is stored in the liver?

A

2-5mg

55
Q

What are some S/S of B12 deficiency?

A

Fatigue
Anorexia
Nausea
Glossitis
Angular cheilitis

56
Q

What are some ways that can cause B12 deficiency?

A

Diet
Decrease intrinsic factor (Pernicious anemia, gastric surgery)
Pancreatic insufficiency
Transcobalamin II deficieny
Medications
Chrohns disease

57
Q

What medications affect B12 absorption?

A

Metformin
PPI
Colchicine

58
Q

What is blind loop syndrome?

A

Bacterial overgrowth of small bowel (fish tapeworm) that competes for B12

59
Q

What are neuro symptoms progress in B12 deficiency? (initial, later, severe)

A

Initial: peripheral paresthesias
Later: balance and proprioception difficulty
Severe: affect cerebral function

60
Q

Why is MCV elevated in B12 deficiency?

A

Due to formation of megaloblasts from impaired DNA synthesis

61
Q

What are present in a peripheral smear for B12 deficiency anemia?

A

Hypersegmented neutrophils
Macro-ovalocytes
Bizarre RBC shapes
Basophilic stippling

62
Q

Whats elevated in testing for pernicious anemia in B12 deficiency?

A

Anti-intrinsic factor antibodies
Anti-parietal cell antibodies
Gastrin levels

63
Q

What tests do we perform on checking pernicious anemia in B12 deficiency?

A

Gastric biopsy
Schilling test (old test using radio labeled B12)

64
Q

What are the treatments for B12 deficiency?

A

Oral supplements 1mg/day
B12 injection therapy
Folic acid therapy 1mg/day
Transfusion
Dietary counseling

65
Q

How fast is B12 deficiency treated?

A

See reticulocytosis in 1 week and normalize in 2 months

66
Q

What are the SE of Cyanocobalamin?

A

HA
Paresthesias
GI upset
Glossitis
Worsening edema or HF
Allergic reaction (parenteral)
Muscle soreness/weakness (parenteral)

67
Q

What do you monitor when pts are on cyanocobalamin?

A

B12
Hgb/Hct
RBC
Reticulocytes
Iron/folate levels

68
Q

What is the role of folate?

A

Coenzyme for conversion of homocysteine to methionine
Involved in DNA synthesis, especially in erythroid precursors

69
Q

How much folate is absorbed and used daily?

A

125mcg/day (50% of eaten 250mcg)
Use 50-100mcg/day

70
Q

What are some causes of folate deficiency?

A

Dietary deficiency (main)
Increased folate requirement
Inhibition of reduction to active form (methotrexate)
Excess folate loss (hemodialysis)
Decreased absorption of folate

71
Q

What can decreased absorption of folate?

A

Tropical sprue
Concurrent B12 deficiency
Phenytion
Sulfasalazine
Trimethoprim-sulfamethoxazole

72
Q

What causes an increase of folate requirement?

A

Pregnancy
Chronic hemolytic anemia
Exfoliative skin disease

73
Q

What is the difference in symptoms of folate and B12 deficiency?

A

Folate deficiency does not have any neuropathy

74
Q

What appears on a peripheral smear for folate deficiency anemia?

A

Hypersegmented neutrophils
Macro-ovalocytes

75
Q

What are some treatments for folic acid deficiency?

A

Oral supplement
Levomethylfolate (active form)
Transfusion

76
Q

What SE appear in taking folic acid?

A

Malaise
Nausea
Allergic reaction symptoms

77
Q

What is a myeloproliferative disorder?

A

Diverse group of disorders categorized by excessive growth of one or more hematopoietic stem cell lines

78
Q

What are the types of myeloprolilferative disorders?

A

Polycythemia vera: excessive production of all hematopoietic cells, especially RBCs
Essential thrombocytosis: excessive platelet production
Myelofibrosis: excessive production of collagen or fibrous tissue in the marrow
Chronic myelogenous leukemia(CML): excessive production of granulocytes

79
Q

What are the S/S of myeloproliferative disorders?

A

Fatigue
Anorexia
Night sweats
Splenomegaly
Hepatomegaly
Superficial vein thrombosis
Bruising
Petechia
Ruddy facial features, plethora (polycythemia vera)

80
Q

What work-ups do you need for myeloproliferative disorders?

A

CBC
Bone marrow biopsy

81
Q

What treatment do all themyeloproliferative disorders have in common?

A

All require myelosuppression
Myelofibrosis and CML may need bone marrow transplant