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
What are some treatments of sideroblastic anemia?
Transfusion B6 (pyridoxine) or B1 (thiamine) Stopping medication (isoniazid) Genetic counseling Phlebotomies, chelation (to prevent iron overload)
26
What is the main cause of anemia worldwide?
Iron deficient anemia
27
How much iron do we need to intake a day?
10-15mg
28
How much iron do we absorb?
10%
29
What is the role of ferroportin?
Iron transporter that release iron from cells
30
What dos Hepcidin do to ferroportin?
Promotes ferroportin breakdown which inhibits iron release
31
How much iron do we lose a day?
Lose 1 mg from skin/mucosa So we absorb ~1mg and lose 1mg
32
What are some causes of iron deficiency anemia?
Deficient diet Increased iron requirements Chronic blood loss Decreased absorption Iron sequestration
33
When do we need an increase of iron consumption?
Pregnancy Lactation Growth spurt
34
What can cause a decreased absorption of iron?
Gastritis Chronic disease Gastric surgery Zinc deficiency Hereditary iron-deficency anemia
35
What are the S/S of iron deficient anemia?
Normal anemic symptoms Smooth tongue Brittle nails Koilonychia Cheilosis Plummer-Vinson syndrome Restless leg Neurodevelopmental delay
36
What is Pica?
Craving for substances not rich in iron (ice, clay, dirt)
37
What are the stages of iron deficiency?
Low iron without anemia Normocytic anemia Microcytic anemia
38
What shows up on a peripheral smear in iron deficiency anemia?
Hypochromic microcytic cells Target cells Poikilocytosis Anisocytosis Increased platelets
39
What are some treatments for iron deficiency anemia?
Supplements (Ferrous sulfate) Transfusion Iron replacement therapy (parenteral, IV)
40
What can you add to increase absorption of ferrous sulfate?
Vitamin C
41
What are the SE and dosage of ferrous sulfate?
325mg 3x/day on empty stomach SE: N/V Constipation Abdominal pain Dark stool Rare SE: Diarrhea Urine discoloration Teeth staining
42
What is contraindicated for ferrous sulfate?
Allergy to drug Hemochromatosis Hemolytic anemia
43
What improved over iron dextran(older parenteral iron)?
New preparations are infused over minutes, not hours Lower risk of serious reactions(anaphylaxis) No risk of "iron staining" (cutaneous siderosis)
44
What causes anemia of inflammation/infection?
Cytokines increased hepcidin which decrease iron absorption and availability Mimics iron deficiency anemia 75% normocytic 25% microcytic
45
Define anemia of CKD.
Anemia due to inadequate secretion of EPO by kidneys Normocytic, normochromic anemia Normal iron Pts on dialysis can develop secondary iron/folate deficiency
46
Define anemia of endocrine disorders.
Decrease EPO secretion Normocytic, normochromic anemia Decreased thyroid, testosterone, cortisol
47
Define anemia of chronic liver disease.
Cholesterol deposits in RBC membrane causing decreased RBC survival and EPO sectretion Macrocytic anemia due to deposits
48
Define Anemia of starvation.
Decreased EPO due to decrease of metabolism(protein intake)
49
Why are elderly susceptible to anemia?
Resistance to EPO Decreased EPO secretion Chronic low-level inflammation
50
What are some treatments for anemia of chronic diseases?
Transfusion EPO (Poetin, darbepoetin)
51
What is the role of vitamin B12(cobalamin)?
Converts methylmalonyl-CoA to succinyl-CoA Converts homocysteine to methionine DNA synthesis in erythroid precursors
52
What are sources of vitamin B12?
Animal-based foods, fortified foods
53
How much B12 do we absorption and use?
Absorb ~5mcg/day Use 3-5 mcg/day
54
How much B12 is stored in the liver?
2-5mg
55
What are some S/S of B12 deficiency?
Fatigue Anorexia Nausea Glossitis Angular cheilitis
56
What are some ways that can cause B12 deficiency?
Diet Decrease intrinsic factor (Pernicious anemia, gastric surgery) Pancreatic insufficiency Transcobalamin II deficieny Medications Chrohns disease
57
What medications affect B12 absorption?
Metformin PPI Colchicine
58
What is blind loop syndrome?
Bacterial overgrowth of small bowel (fish tapeworm) that competes for B12
59
What are neuro symptoms progress in B12 deficiency? (initial, later, severe)
Initial: peripheral paresthesias Later: balance and proprioception difficulty Severe: affect cerebral function
60
Why is MCV elevated in B12 deficiency?
Due to formation of megaloblasts from impaired DNA synthesis
61
What are present in a peripheral smear for B12 deficiency anemia?
Hypersegmented neutrophils Macro-ovalocytes Bizarre RBC shapes Basophilic stippling
62
Whats elevated in testing for pernicious anemia in B12 deficiency?
Anti-intrinsic factor antibodies Anti-parietal cell antibodies Gastrin levels
63
What tests do we perform on checking pernicious anemia in B12 deficiency?
Gastric biopsy Schilling test (old test using radio labeled B12)
64
What are the treatments for B12 deficiency?
Oral supplements 1mg/day B12 injection therapy Folic acid therapy 1mg/day Transfusion Dietary counseling
65
How fast is B12 deficiency treated?
See reticulocytosis in 1 week and normalize in 2 months
66
What are the SE of Cyanocobalamin?
HA Paresthesias GI upset Glossitis Worsening edema or HF Allergic reaction (parenteral) Muscle soreness/weakness (parenteral)
67
What do you monitor when pts are on cyanocobalamin?
B12 Hgb/Hct RBC Reticulocytes Iron/folate levels
68
What is the role of folate?
Coenzyme for conversion of homocysteine to methionine Involved in DNA synthesis, especially in erythroid precursors
69
How much folate is absorbed and used daily?
125mcg/day (50% of eaten 250mcg) Use 50-100mcg/day
70
What are some causes of folate deficiency?
Dietary deficiency (main) Increased folate requirement Inhibition of reduction to active form (methotrexate) Excess folate loss (hemodialysis) Decreased absorption of folate
71
What can decreased absorption of folate?
Tropical sprue Concurrent B12 deficiency Phenytion Sulfasalazine Trimethoprim-sulfamethoxazole
72
What causes an increase of folate requirement?
Pregnancy Chronic hemolytic anemia Exfoliative skin disease
73
What is the difference in symptoms of folate and B12 deficiency?
Folate deficiency does not have any neuropathy
74
What appears on a peripheral smear for folate deficiency anemia?
Hypersegmented neutrophils Macro-ovalocytes
75
What are some treatments for folic acid deficiency?
Oral supplement Levomethylfolate (active form) Transfusion
76
What SE appear in taking folic acid?
Malaise Nausea Allergic reaction symptoms
77
What is a myeloproliferative disorder?
Diverse group of disorders categorized by excessive growth of one or more hematopoietic stem cell lines
78
What are the types of myeloprolilferative disorders?
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
What are the S/S of myeloproliferative disorders?
Fatigue Anorexia Night sweats Splenomegaly Hepatomegaly Superficial vein thrombosis Bruising Petechia Ruddy facial features, plethora (polycythemia vera)
80
What work-ups do you need for myeloproliferative disorders?
CBC Bone marrow biopsy
81
What treatment do all themyeloproliferative disorders have in common?
All require myelosuppression Myelofibrosis and CML may need bone marrow transplant