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Hem/Onc > Anemias- Part II > Flashcards

Anemias- Part II Flashcards

1
Q

What is aplastic anemia caused by?

A

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

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2
Q

What is the MC cause of aplastic anemia?

A

idiopathic autoimmune suppression of hematopoiesis

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3
Q

What diseases are associated with aplastic anemia?

A

Paroxysmal nocturnal hemoglobinuria, lupus, transfusion-related graft-versus-host disease

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4
Q

benzene, toluene, insecticides, mercury are toxins associated with _____

A

aplastic anemia

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5
Q

chemo, anticonvulsants (phenytoin, carbamazepine), chloramphenicol, cimetidine, sulfa drugs are all medications associated with ______

A

aplastic anemia

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6
Q

What infections are associated with aplastic anemia?

A

hepatitis, Epstein-Barr virus, parvovirus B19, cytomegalovirus

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7
Q

Name some additional causes of aplastic amemia

A

radiation exposure, pregnancy, congenital

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8
Q

In what kind of anemia does hypoplasia of hematopoietic bone marrow leads to decrease in all hematopoietic cell lines?

A

aplastic anemia

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9
Q

Decreased WBC in aplastic anemia is likely due to _____

A

infections

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10
Q

What s/s would you expect to find in a pt with aplastic anemia with decreased RBC

A

pallor, fatigue, dyspnea, palpitations

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11
Q

Decreased platelets in aplastic anemia will present with ____ and _____

A

bruising and bleeding

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12
Q

What would you expect to find on a bone marrow biopsy for an aplastic anemia pt?

A

hypocellular aspirate; limited or no hematopoietic precursors

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13
Q

What are the supportive treatments for a pt with aplastic anemia?

A

red cell transfusions, platelet transfusions, antimicrobials

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14
Q

_____ Initially developed for thrombocytopenia, but shown to also boost RBC and WBC production in _____ patients?

A

eltrombopag (Promacta)

aplastic anemia

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15
Q

What are the three medication treatment options for mild/moderate aplastic anemia?

A

eltrombopag (Promacta)
epoetin, darbepoetin
filgrastim, sargramostim

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16
Q

What is considered severe aplastic anemia?

A

neutrophil count (ANC) <500/mcL

platelets <20,000/mcL

absolute reticulocyte count (ARC) <60,000/mcL

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17
Q

What is considered very severe aplastic anemia?

A

ANC <200/mcL (neutrophil count)

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18
Q

What is the treatment for a severe aplastic anemia pt who is less than 40 years old?

A

Bone marrow transplant - pts <40 with fully HLA-matched sibling donor

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19
Q

What is the treatment for a severe aplastic anemia pt who is older than 40?

A

Immunosuppression (Triple IST) - 40+ y/o or can’t undergo transplant

Horse/Equine antithymocyte globulin (ATG)
–Steroids often given along with ATG to reduce side effects

cyclosporine - interleukin-II inhibitor

eltrombopag (Promacta) - bone marrow stimulating drug

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20
Q

What is the brand name for epoetin alfa?

A

Epogen, Procrit

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21
Q

What is the brand name for darbepoetin alfa?

A

Aranesp

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22
Q

What is the MOA for Epoetin / Darbepoetin?

A

human erythropoietin (EPO) made via recombinant DNA

Stimulates division and differentiation of erythroid precursors → reticulocyte and RBC release

Eventual rise in Hb and Hct (peaks in 2-6 weeks); dose-dependent

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23
Q

Which severe aplastic anemia drug has a 1/2 life that is 3x longer than the other?

A

Darbepoetin has a half-life about 3x that of epoetin alfa

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24
Q

What is Epoetin / Darbepoetin indicated for?

A

Anemia due to CKD, chemotherapy, myelodysplasia

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25
Q

What are the contraindications of Epoetin / Darbepoetin? What is the major one?

A

allergy to rx; uncontrolled HTN; pure red cell aplasia after any EPO tx

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26
Q

What are the black box warnings associated with Epoetin / Darbepoetin?

A

↑ risk of death, MI, stroke, venous thromboembolism, thrombosis of vascular access

↑ risk of death, cardiovascular reactions, and stroke in CKD pts with Hb >11

↑ risk of death and/or tumor progression for certain types of cancers

↑ risk of DVT after surgery - DVT prophylaxis needed for surgical pts on EPO

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27
Q

What are the side effects associated with Epoetin / Darbepoetin? What are the major 2?

A

HTN, thrombosis, rash, seizures, pruritus, fever, edema, dyspnea, cough, abdominal pain

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28
Q

What parameters do you think we would want to monitor on a pt that is taking Epoetin / Darbepoetin?

A

CBC, iron status, BLOOD PRESSURE!

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29
Q

_____ Mixed group of disorders that share abnormalities in heme synthesis and mitochondrial function. What is common among all of them?

A

Sideroblastic anemia

ring sideroblasts in bone marrow aspirate

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30
Q

______ decreased Hb synthesis 2o
reduced ability to synthesize heme
because of impaired ability to
incorporate iron into protoporphyrin
IX (precursor to heme)

A

Sideroblastic anemia

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31
Q

What are the types of inheritance associated with sideroblastic anemia? Is it more common in males or females?

A

X-linked (most common of inherited forms)
Autosomal recessive
Mitochondrially inherited

slightly more common in males

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32
Q

What are some causes of acquired sideroblastic anemia?

A

Often part of a general myelodysplastic syndrome

Chronic alcoholism

Lead poisoning

Copper deficiency

Chronic infection/inflammation

Medications: mostly antimicrobials
isoniazid, linezolid, chloramphenicol

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33
Q

Is acquired or genetic sideroblastic anemia more common?

A

acquired is more common

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34
Q

How does sideroblastic anemia present?

A

fatigue, tachycardia, dizziness, dyspnea on exertion, palpitations
Exam - pallor of conjunctiva, palmar creases (if Hb <8-9)

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35
Q

What is myelodysplastic syndrome?

A

a group of cancers that cause immature blood cells in the bone marrow to not mature or become healthy

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36
Q

In what type of anemia does the body have plenty of iron but it cannot stick it in the Hgb?

A

Sideroblastic anemia

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37
Q

What lab values are INCREASED in a pt with sideroblastic anemia?

A

RDW
Transferrin saturation
Iron
Ferritin

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38
Q

What kind of inclusion is associated with sideroblastic anemia?

A

basophilic stippling

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39
Q

What lab test MUST be ordered in a pt with sideroblastic anemia?

A

bone marrow aspirate

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40
Q

What does Erythroid hyperplasia indicate?

A

indicates ineffective erythropoiesis

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41
Q

What does Prussian Blue Stain show? What type of anemia?

A

hows ringed sideroblasts (erythroid cells with iron deposits in mitochondria encircling the nucleus) and general increase in iron stores

sideroblastic anemia

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42
Q

What are some treatments for sideroblastic anemia?

A

Transfusions: for severe

Some forms will respond to B6 and B1

Stopping medication that caused it, if drug induced

phlebotomies for iron overloaded pts

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43
Q

What is the MC cause of anemia worldwide?

A

Iron deficiency anemia

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44
Q

What is the normal daily dietary iron needed?

A

10-15mg iron

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45
Q

XX: What is the percentage breakdown of iron for meat? Heme vs nonheme, % absorbed?

A

Meat: 40% heme/ 60% nonheme- 10-20% absorbed

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46
Q

What type of iron is the iron found in vegetables? What percent is absorbed?

A

Nonheme is all vegetable iron

1-5% absorbed

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47
Q

In a normal acidic environment, what percentage of iron is absorbed?

A

10% overall absorption

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48
Q

_____ - major iron transporter - releases iron from cells

A

Ferroportin

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49
Q

_____ promotes ferroportin breakdown → inhibits iron release

A

Hepcidin

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50
Q

_____ amount of iron is lost through the skin/mucosa daily

A

1mg/day

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51
Q

In iron deficiency anemia, what are the causes of iron loss?

A

deficient diet
increased requirements
chronic blood loss

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52
Q

What things would cause the iron requirements to increase?

A

pregnancy, lactation, growth spurt

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53
Q

Name 6 examples of chronic blood loss.

A

Menstrual - May need up to 3-4 mg/day of iron if heavy menses

GI - peptic ulcers, IBD, cancer, chronic ASA/NSAID use

Regular blood donation/phlebotomy

Chronic intravascular hemolysis/hemoglobinuria - chronic iron loss

decreased iron absorption

iron sequestration

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54
Q

What is Chronic intravascular hemolysis/hemoglobinuria?

A

chronic iron loss

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55
Q

Name some causes of decreased iron absorption

A

*Gastritis - H. pylori-related, autoimmune gastritis

*Chronic disease - Celiac sprue or Crohn’s disease

Gastric surgery

Zinc deficiency

Hereditary iron-deficiency anemia

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56
Q

What are some s/s of iron deficiency anemia?

A

fatigue, tachycardia, dizziness, dyspnea on exertion, palpitations, smooth tongue, brittle nails, koilonychia, cheilosis, restless leg syndrome, neurodevelopmental delay, pica, Plummer-Vinson syndrome

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57
Q

What is Plummer-Vinson Syndrome?

A

esophageal webs leading to dysphagia

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58
Q

What is pica?

A

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

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59
Q

What is koilonychia?

A

spooned nails

60
Q

An anemic pt should have a (high/low) retic count

A

high retic count

61
Q

An iron deficiency anemia pt what will their iron studies look like?

A

Total Iron Binding Capacity (TIBC) - increased

Transferrin saturation - decreased

Iron - decreased

Ferritin - decreased
< 12 (no anemia) or <30 (anemic)

62
Q

An iron deficiency anemia pt will have (increased/decreased) platelets

A

increased

63
Q

What is first line treatment for iron deficiency anemia? What can you give with it to increase absorption? How much is actually absorbed?

A

Ferrous sulfate 325 mg orally three times per day on an empty stomach

ascorbic acid

65/325

64
Q

How long do you need to continue supplemental iron after anemia has resolved?

A

6 months to build up iron stores

65
Q

What is given to an iron deficiency anemia pt who cannot tolerate ferrous sulfate?

A

Ferrous gluconate 39/325 is absorbed

66
Q

What is the MOA for ferrous sulfate?

A

replaces iron found naturally in the human body
In setting of iron deficiency anemia, enables marrow to engage in erythrocyte production

67
Q

What are the SE of ferrous sulfate?

A

N/V, constipation, dark stools, abdominal pain/cramping

May more rarely see diarrhea, discoloration of urine, teeth staining

68
Q

What do you want to monitor on a pt taking ferrous sulfate?

A

iron, TIBC, ferritin, retic count, Hgb/Hct/RBC

69
Q

For a severely iron deficient anemia pt, what is the treatment?

A

transfusions

IV Iron replacement

70
Q

What kind of patients are better candidates for IV iron instead of oral?

A

Hx of bariatric surgery, GI malabsorption

May also be used for late-stage renal disease, later in pregnancy

71
Q

What is cutaneous siderosis?

A

iron staining

72
Q

_____ is the older form of parenteral iron, IM form - still had risk of anaphylaxis, “iron staining”

A

Iron dextran

73
Q

ferric carboxymaltose, ferumoxytol (Feraheme), iron sucrose (Venofer), sodium ferric gluconate (Ferrlecit) all fall under what category?

A

newer preparations of IV iron

74
Q

What is the cause of anemia of chronic disease?

A

proinflammatory cytokines → increased hepcidin → decreased iron absorption and availability

75
Q

How does anemia of inflammation/infection present?

A

May mimic iron deficiency anemia
Normocytic (75%) or microcytic (25%)
Decreased iron levels

76
Q

How do you think we could differentiate anemia of inflammation from anemia due to iron deficiency?

A

Anemias of chronic disease with have increased/normal ferritin levels

iron deficiency anemia pt will have LOW ferritin

77
Q

What is the cause of anemia of chronic kidney disease?

A

Failure to secrete adequate EPO by kidneys
Severity of anemia correlates with severity of CKD

78
Q

How does Anemia of Chronic Kidney Disease present?

A

Known hx of CKD anemic symptoms
Normocytic, normochromic anemia
Normal iron studies in most patients
Pts on dialysis may develop a secondary iron and/or folate
deficiency, complicating their anemia

79
Q

Decreased EPO secretion → normocytic, normochromic anemia are seen with what kind of anemia?

A

Anemia of Endocrine Disorders

80
Q

Cholesterol deposits in RBC membrane → shortened RBC survival and inadequate EPO secretion to compensate are seen with what kind of anemia? Often seen in what kind of pts? What happens to the size of the RBC?

A

Anemia of Chronic Liver Disease

alcoholics

Increased in RBC size, macrocytic anemia

81
Q

resistance to EPO, decreased EPO secretion, and chronic low-level inflammation is found in what kind of anemia?

A

Anemia of the elderly?

82
Q

Anemia of the elderly is found in what percentage of ????

A

Up to 20% of pts >85 y/o

83
Q

What is the treatment for anemias of chronic disease?

A

Correction or management of underlying cause

Transfusions - as indicated by labs and presentation

EPO - epoetin or darbepoetin may be helpful for severe anemia in states where EPO secretion is diminished

84
Q

What is the role of Vit B12 in the body? Why is this important?

A

Converts methylmalonyl-CoA to to succinyl-CoA
Converts homocysteine to methionine

Both steps involved in DNA synthesis, especially in erythroid precursors

85
Q

What is the daily absorption of vit B12? How much is utilized per day?

A

5 mcg/day

3-5mcg/day

86
Q

B12 normally binds to _____ in the stomach

Where is it absorbed?

Where is it stored?

A

intrinsic factor

terminal ileum

liver (2-5mg stored)

87
Q

What are some causes of B12 deficiency

A

dietary deficiency

decreased intrinsic factor

pancreatic insufficiency

transcobalamin II deficiency

medications

competition for B12

decreased absorption

88
Q

What 3 types of pts are commonly dietary deficient in B12

A

vegan

alcoholics

elderly

89
Q

What two factors can contribute to decreased intrinsic factor

A

Pernicious anemia - autoimmune Ig to gastric parietal cells, intrinsic factor, or both

Gastric surgery - especially for weight loss

90
Q

What three medications are associated with B12 deficiency?

A

metformin, proton pump inhibitors, colchicine

91
Q

fish tapeworms can cause ????

A

competition for B12 and B12 deficiency

92
Q

What is blind loop syndrome?

A

bacterial overgrowth of small bowel

93
Q

What is Transcobalamin II?

A

protein that transports B12

94
Q

What does a B12 deficiency present with?

A

Anemia (maybe with decreased WBC and platelets)
anorexia, nausea, glossitis, angular cheilitis
fatigue, gradual onset of neuropathy

95
Q

What is the neuro symptoms manifestation timeline in B12 deficiency?

A

Initial - peripheral paresthesias
Later - difficulty with balance and/or proprioception
Severe - may affect cerebral function

96
Q

hypersegmented neutrophils consisting of 8-10 lobes are seen in the peripheral smear of what kind of anemia?

A

B12 deficiency

97
Q

B12 deficiency anemia has what kind of inclusion body?

A

basophilic stippling

98
Q

What tests can be ordered to test for pernicious anemia?

A

Anti-intrinsic factor antibodies
Anti-parietal cell antibodies
Gastrin levels
Gastric biopsy
Schilling test

99
Q

What is the Schilling test?

A

old test used to identify PA as cause of B12 deficiency
Administered B12 IM injection, oral radiolabeled B12, +/- oral intrinsic factor, and measured urine output of radiolabeled B12

100
Q

What are the different injectable supplements for B12 deficiency?

A

Cyanocobalamin (synthetic B12)

Hydroxocobalamin (synthetic B12a)

101
Q

Why would you use Cyanocobalamin (synthetic B12) vs Hydroxocobalamin?

A

Cyanocobalamin (synthetic B12) is cheaper and first line

Hydroxocobalamin has a longer 1/2 life so the patient can go longer in between doses

102
Q

What is the antidote for cyanide poisoning?

A

Hydroxocobalamin

103
Q

When would you use an injectable vs oral B12 supplemental therapy?

A

injectable for pernicious anemia because the pt’s body cannot absorb B12 normally

May use oral or sublingual B12 if mild (Hgb >8) and no neuro s/s, or for maintenance
If absorption is adequate orally

104
Q

______ is the naturally occurring form of B12 found in foods. Is it found in an injectable form?

A

Methylcobalamin

NOT found in an injectable form

105
Q

What is the treatment for B12 deficiency?

A

B12 supplementation

folic acid therapy: 1 mg/day

Transfusions (rarely needed)

dietary counseling

106
Q

When should you begin to see a response from treatment for a pt with B12 deficiency in terms of retic count and CBC?

A

retic: see improvement in 1 week

CBC: 2 months

107
Q

What are the SE of Cyanocobalamin? parenteral form?

A

headache; paresthesias; GI upset; glossitis

allergic reaction, muscle soreness/weakness

108
Q

What labs do you need to monitor on a pt on cyanocobalamin?

A

B12 level, Hb/Hct, RBCs, reticulocytes, iron and folate levels

Maintenance monitoring - CBC and B12 level every 3-6 months chronically

109
Q

What is the role of folate in the body?

A

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

110
Q

What is the main cause of folic acid deficiency? Due to ????

A

dietary deficiency due to anorexia, alcoholism, no fruits/vegetables, overcooked fruits/vegetables

111
Q

Under what 3 conditions would you need to increase your folate requirements?

A

Pregnancy
Chronic hemolytic anemia
Exfoliative skin disease

112
Q

Besides dietary deficiencies, what are 3 additional causes of folate deficiency?

A

Inhibition of reduction to active form (methotrexate)
Excess folate loss (hemodialysis)
Decreased absorption of folate

113
Q

When writing for methotrexate, you should also write for _____ concurrently

A

folate

114
Q

decreased absorption of folate can be due to ????

A

Tropical sprue

Concurrent vitamin B12 deficiency

Medications - phenytoin, sulfasalazine, trimethoprim-sulfamethoxazole

115
Q

Where is iron absorbed?

A

duodenum

116
Q

Where is folate absorbed?

A

jejunum

117
Q

Where is B12 absorbed?

A

Ileum

118
Q

What does a folate deficiency present like?

A

moderate to severe; insidious onset
—-May also see ↓ WBC and platelets

anorexia, nausea, glossitis, angular cheilitis
fatigue

NO neuropathy

119
Q

If I am concerned about a folate or B12 deficiency, what 2 not normally ordered labs should you order?

A

Homocysteine

Methylmalonic acid

120
Q

What is the folic acid replacement recommended dose?

A

Oral folic acid, 1-5 mg/daily for at least 4 months

121
Q

If you are thinking about a folic acid deficiency, you must also rule out ______

A

B12 deficiency

122
Q

________ is the activated/metabolized form of folate

A

Levomethylfolate (L-methylfolate)

123
Q

What are the SE of folate?

A

malaise, nausea

124
Q

What are the allergic reaction s/s of folate?

A

malaise, flushing, erythema, rash, bronchospasm

125
Q

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

A

Myeloproliferative Disorders

126
Q

_____ excessive production of all hematopoietic cells, especially RBCs

A

Polycythemia vera

127
Q

_____ excessive platelet production

A

Essential thrombocytosis

128
Q

____ excessive production of collagen or fibrous tissue in the marrow

A

myelofibrosis

129
Q

_____ excessive production of granulocytes

A

Chronic myelogenous leukemia (CML)

130
Q

How do Myeloproliferative Disorders present like?

A

fatigue, anorexia, weight loss, night sweats

splenomegaly, hepatomegaly, abdominal discomfort, early satiety

pallor, easy bruising, petechiae, bleeding, superficial vein thrombosis

131
Q

In polycythemia vera, instead of pallor, patients often have _____

A

ruddy facial features (plethora)

132
Q

What is plethora?

A

ruddy facial features

133
Q

How are Myeloproliferative Disorders
associated with anemias?

A

Abnormal cell growth competes with erythroid precursors for space, nutrients

Erythroid precursors often are suppressed or damaged, limiting normal function

134
Q

If you are concerned about a Myeloproliferative Disorders
what labs should you order?

A

CBC - will detect anemia (if present), along with WBC and platelet abnormalities

Bone marrow biopsy - helps delineate presence of abnormal cells in marrow

135
Q

How do you treat Polycythemia vera?

A

regular phlebotomies; myelosuppression

136
Q

How do you treat Essential thrombocytosis?

A

observation if asymptomatic; myelosuppression

137
Q

How do you treat Myelofibrosis?

A

observation if asymptomatic; myelosuppression; bone marrow transplant

138
Q

How do you treat CML?

A

chemotherapy; myelosuppression; bone marrow transplant

139
Q

_____ increased total-body iron, usually caused by a genetic variant leading to increased intestinal iron absorption

A

Hemochromatosis

140
Q

When does Hemochromatosis usually clinically manifest?

A

40+

141
Q

How does Hemochromatosis present?

A

Fatigue, widespread pain, weakness, lethargy

Skin deposition - “bronze” hyperpigmentation

Liver deposition - abnormal liver function tests, hepatomegaly, cirrhosis, cancer

Heart deposition - cardiomegaly, heart failure

Endocrine deposition - diabetes mellitus, hypogonadism

142
Q

What is the classic triad associated with Hemochromatosis?

A

hyperpigmentation, DM, cirrhosis (“bronze diabetes”)

143
Q

Hemochromatosis affects the ____ the most

A

liver

144
Q

What are common lab findings on a pt with Hemochromatosis?

A

Iron studies - reflective of iron overload

Elevated iron, ferritin, and transferrin saturation

Low TIBC

Liver function tests - usually reflective of liver injury

145
Q

What is the treatment for Hemochromatosis?

A

Routine phlebotomy

Iron chelation - may be considered, but usually not first-line

Hem/Onc (14 decks)

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