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Heme/Onco > Erythrocyte Disorders > Flashcards

Erythrocyte Disorders Flashcards

1
Q

Anemia

What are 3 main causes?

A
  1. Underproduction
  2. Destruction
  3. Blood loss
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2
Q

Anemia

How to differentiate between underproduction and destruction?

A

Reticulocyte

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3
Q

Anemia

What is the corrected reticulocyte formulation?

A

Reticulocyte production index

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4
Q

Anemia

What does MCV stand for?

A

Differentiate microcytic and macrocytic

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5
Q

Anemia

Anemia with MCV <80fL?

A
  1. Iron deficiency;

2. Thalassemia

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6
Q

Anemia

Anemia with MCV wnl?

A
  1. Inflammatory anemia;
  2. Anemia of kidney disease;
  3. Hereditory spherocytosis;
  4. Sickle cell disease
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7
Q

Anemia with MCV>100?

A
  1. Vitamin B12 or folate deficiency;
  2. Autoimmune hemolytic anemia;
  3. Liver disease;
  4. Hypothyroidism;
  5. Myelodysplastic syndrome;
  6. Sideroblastic anemia;
  7. Alcohol;
  8. Drugs (HU, ZDV)
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8
Q

Iron deficiency anemia

Where is iron absorbed in the GI?

A

Duodenum

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9
Q

Iron deficiency anemia

What is hepcidin?

A

It is a peptide hormone produced in the liver, main regulator of iron homeostasis.

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10
Q

Iron deficiency anemia

How does hepcidin work?

A

Decreases intestinal iron absorption and release of iron stores.

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11
Q

Iron deficiency anemia

When is hepcidin produced?

A

When iron is abundant and in inflammatory anemia.

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12
Q

Iron deficiency anemia

When is hepcidin suppressed?

A

In iron deficiency anemia.

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13
Q

Iron deficiency anemia

What are the common causes?

A
  1. Loss of iron from bleeding: menstruation; phlebotomy, GI or GU bleeding
  2. Decreased intake:
  3. Decreased absorption: after gastric/duodenal surgery; celiac disease; H.pylori infection; autoimmune atrophic gastritis
  4. Increased iron requirement: pregnancy and lactation
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14
Q

Iron deficiency anemia

Clinical manifestations:

A

SOB, dizziness, fatigue, pica (ice, dirt, clay, paper, and laundry starch);
Restless leg syndrome;
Hair loss.

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15
Q

Iron deficiency anemia

What is transferrin saturation?

A

Serum iron/TIBC

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16
Q

Iron deficiency anemia

What is the value of transferrin saturation?

A

<15%

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17
Q

Iron deficiency anemia

Value of ferritin?

A

<=12ng/mL (ug/L)

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18
Q

Iron deficiency anemia

How to differentiate IDA from inflammatory anemia?

A

Ferritin can be normal if both IDA and inflammatory anemia.

Ferritin will be >100 exclude IDA.

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19
Q

Iron deficiency anemia

If in premenopausal women what is the cause?

A

Usually from menstrual blood loss.

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20
Q

Iron deficiency anemia

If older men and women what is the cause?

A

Very likely colon cancer or premalignant polyps–>colonoscopy

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21
Q

Iron deficiency anemia
Rx
What are 3 ways?

A
  1. PRBC
  2. Oral replacement
  3. IV replacement
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22
Q

Iron deficiency anemia

Rx-how much iron does 1 unit of blood contain?

A

225-250mg of iron

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23
Q

Iron deficiency anemia

Rx-how much iron dose oral supplement contain?

A

Ferrous sulfate-65mg per 325mg tablet (least expensive)
Ferrous gluconate-36mg per 300-mg tablet
Ferrous fumarate-33mg per 100-mg tablet

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24
Q

Iron deficiency anemia

Rx-What is the indication of oral supplement?

A

IDA not due to impaired intestinal absorption.

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25
Q

Iron deficiency anemia

How to test if there is GI malabsorpation of Iron supplement?

A

Oral challenge:

  1. Check fasting serum iron level;
  2. Give 60mg of oral element iron
  3. Check serum iron again after 1-2 hours
  4. Increase>100ug/dL suggesting adequate oral absorption
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26
Q

Iron deficiency anemia

Rx-How much oral iron need to give per day?

A

Total 150-200mg of iron divided by 2 or 3 times per day

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27
Q

Iron deficiency anemia

Rx- What is the indication of PRBC?

A
  1. severe, symptomatic anemia
  2. HD unstable patients
  3. End-organ damage
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28
Q

Iron deficiency anemia

Rx- What are the side effects of oral supplement?

A

N/V, constipation, black stool, metallic taste

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29
Q

Iron deficiency anemia

DDx- When iron deficiency refractory to iron PO supplement?

A
  1. Subclinical H.pylori infection–>treat infection

2. Subclinical hypothyroidism–>give supplement of levothyroxine

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30
Q

Iron deficiency anemia

Rx-what is the indication for IV replacement?

A
  1. GI malabsorption;
  2. Inability to tolerate PO iron;
  3. Poor response to oral iron;
  4. Patients undergoing kidney dialysis receiving ESAs
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31
Q

Iron deficiency anemia

Rx- what are the formulation if IV iron?

A
  1. Ferric gluconate: 12.5mg/mL
  2. Iron dextran: 50mg/mL
  3. Iron sucrose: 20mg/mL
  4. Ferumoxytol: 30mg/mL
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32
Q

Inflammatory anemia

What it the labs characteristics?

A

Low TIBC <250;
High ferritin >35;
Low serum iron <60;

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33
Q

Anemia of kidney disease

What is the RBC morphology?

A

Normochromic;
Normocytic;
Can see burr cells

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34
Q

Anemia of kidney disease

What is the role of EPO level in dx?

A

NOT useful

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35
Q

Vitamin B12 deficiency

What are 2 other chemicals increased?

A
  1. Methylmalonic acid (MMA);

2. Homocystein levels.

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36
Q

Vitamin B12 deficiency

What is the morphology change of blood cells?

A
  1. Macrocytosis;
  2. Hemolysis of erythrocytes within the bone marrow;
  3. Hypersegmentation in granulocytes
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37
Q

Vitamin B12 deficiency

What are causes?

A
  1. Gastric,bariatric, or ileal surgery;
  2. IBD;
  3. pernicious anemia
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38
Q

Vitamin B12 deficiency

How long dose it take to be deficient?

A

2-3 years

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39
Q

Vitamin B12 deficiency

Clinical manifestation

A 
Anemia;
Hemolysis;
Neurologic dysfunction: numbness, decreased vibratory sense, gait problems, neuropsychiatric symptoms (can present without anemia)
Glossitis;
Hyperpigmentation;
Infertility
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40
Q

Vitamin B12 deficiency

Pernicious anemia, what other comorbidities?

A

Vitiligo;
DM;
Thyroid disease.

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41
Q

Vitamin B12 deficiency

What peripheral blood smear show?

A

Large oval erythrocytes;
Hypersegmented neutrophils (>6 lobes);
Pancytopenia.

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42
Q

Vitamin B12 deficiency

Dx-first step?

A

Serum cobalamin level

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43
Q

Vitamin B12 deficiency
Dx
What is the serum cobalamin level indicating unlikely deficiency?

A

> 300pg/mL (221 pmol/L)

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44
Q

Vitamin B12 deficiency
Dx
What is the serum cobalamin level indicating very likely deficieny?

A

<200pg/mL

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45
Q

Vitamin B12 deficiency

What is the senstivity and sepicificity of measuring Cobalamin level

A

very poor

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46
Q

Vitamin B12 deficiency

When should level be checked?

A

Before starting treatment

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47
Q

Vitamin B12 deficiency
Dx-
What else to measure?

A

MMA and Homocysteine

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48
Q

Vitamin B12 deficiency
Dx-
What level of MMA indicating deficiency?

A

MMA>500nmol/L

can increase in AKI

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49
Q

Vitamin B12 deficiency
Dx-
Is bone marrow biopsy needed?

A

No

It is confusing. Only when not responding

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50
Q

Vitamin B12 deficiency
Dx-
When is paritel cell antibody elevated?

A
  1. Pernicious anemia

2. Chronic gastritis

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51
Q

Vitamin B12 deficiency
Dx-
Pernicious anemia

A

Intrinsic factor antibody

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52
Q

Vitamin B12 deficiency
Rx:
Initiating step

A

High-dose PO 1000-2000 ug/d even in intrinsic factor insufficiency

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53
Q

Vitamin B12 deficiency
Rx:
When to start IM or IV supplement?

A
  1. Severe anemia;
  2. Neurologic dysfunction;
  3. Not responding to PO replacement
54
Q

Vitamin B12 deficiency
Rx
IM management

A
  1. 1000 ug several times per week x 1-2 weeks;
  2. Then weekly until symptoms improved;
  3. Then monthly if underlying causes persists (pernicious anemia)
55
Q

Vitamin B12 deficiency
F/u
When to check Reti count?

A

1 week

56
Q

Vitamin B12 deficiency

Who is at risk?

A

Vegetarians

57
Q

Folate Deficiency

Who are at risk?

A

Older adults, nursing homes, alcoholics

58
Q

Folate Deficiency

Medication causing folate deficiency?

A

Phenytoin;
Trimethoprim;
MTX

59
Q

Folate Deficiency

Diseases causing deficiency?

A

IBD, short gut syndrome, celiac disease

60
Q

Folate Deficiency

High demand causing deficiency?

A

Pregnancy;
Lactation;
States of chronic hemolysis;
Exfoliant dermatitis

61
Q

Folate Deficiency

Dx

A

Folate; (good screening tool, can fluctuate with meals)
High homocysteine
MMA wnl

62
Q

Folate Deficiency

Rx

A

PO folic acid

5mg daily until complete hemotologic recovery

63
Q

Folate Deficiency
Rx
Is PO supplement suffcient in malabsorption?

A

Yes

64
Q

Folate Deficiency

What need to r/o before Rx?

A

B12 deficiency

Because it will make them more vulnerable to central and peripheral nervous symptoms

65
Q

Thalassemia

Normal Hemoglobin

A

Hemoglobin A (alpha2beta2)

66
Q

Thalassemia

Hemoblogin A2

A

alpha2delta2

67
Q

Thalassemia

Hemoblogin F

A

alpha2gama2

68
Q

Thalassemia

alpha-thalassemia

A

Duplication of the alpha-blobin chain on chromosome 16 results 4 alph-globins genes (aa/aa)

69
Q

Thalassemia
alpha-thalassemia
If absence of 1 alpha gene
Sx:

A

(-a/aa)

Asymptomatic carrier state

70
Q

Thalassemia
alpha-thalassemia
If absence of 2 alpha gene
Sx

A

(–/aa) (-a/-a)

Mild microcytic anemia

71
Q

Thalassemia
alpha-thalassemia
If absence of 3 alpha gene
Sx:

A

(–/-a) Hemoglobin H disease

Hemolysis, splenomegaly

72
Q

Thalassemia
alpha-thalassemia
If absence of 4 alpha gene
Sx:

A

Hydrops fetalis and intrauterine fetal demise

73
Q

Thalassemia
Beta-thalassemia
Categories

A

Thalaseemia minor, intermedia, and major

74
Q

Thalassemia
Beta-thalassemia
Minor mutations:

A

Heterozygous gene mutation

75
Q

Thalassemia
Beta-thalassemia
Minor Sx

A

Asymptomatic, microcytic mild anemia

76
Q

Thalassemia
Beta-thalassemia
Intermedia Sx

A

Moderate hemolytic anemia, Hgb>7 without transfusion support;
Worsening anemai if aplastic crisis during infection

77
Q

Thalassemia
Beta-thalassemia
Intermedia Complications

A

Chronic hemolysis, folate deficiency, chlelithiasis

78
Q

Thalassemia
Beta-thalassemia
Major Sx:

A

Early life with pallor, failure to thrive, severe hemolytic anemia, erythroid hyperplasia in the bone marrow, bone defomities, massive HSM, extramedullary hematopoiesis

79
Q

Thalassemia
Beta-thalassemia
Rx:

A
  1. Transfusion if Hgb<7

2. Transplant can be curative.

80
Q

Thalassemia
Beta-thalassemia
Complications

A

Iron overload –>caridomyopathy, liver fibrosis, endocrinology problem;

81
Q

Thalassemia
Beta-thalassemia
Rx of iron overload

A

SQ desferrioxamine or PO deferasirox, deferiprone

82
Q

Sickle cell anemia

Rx strategies

A
  1. Transplant
  2. Prophylatic transfusion
  3. Hydroxyurea
83
Q

Sickle cell anemia

When to give hydroxyurea

A

> =1-2 episodes of vaso-occlusive episodes

84
Q

Hemolytic anemia

Other causes of hemolysis

A

Macroangiopathic hemolytic anemia
March hemoglobinuria
Hemolysis associated with chemicals and physical agents
Hemolysis from infections

85
Q

Hemolytic anemia
Macroangiopathic hemolytic anemia
Etiology

A

Heart valves (mechanical heart valve)
Intracardiac tumors
Rupture of chordae tendinea

86
Q

Hemolytic anemia
Macroangiopathic hemolytic anemia
Lab findings

A

Schistocytes

87
Q

Hemolytic anemia
March Hemoglobinuria
Etiology

A

Repetitive trauma to soles or palms, seen in marching, ranning, drum playing, karate or head baning

88
Q

Hemolytic anemia
March hemoglobinuria
Lab findings

A

Positive urine hemosiderin test

89
Q

Hemolytic anemia

Hemolysis associated with chemical and physical agents

A

Arsenic
Elevated copper (Wilson disease)
Insect and spider bites (Brown recluse spider)
Severe burns (direct thermal injury to RBCs)

90
Q

Hemolytic anemia
Hemolysis from infections
Causes

A

Plasmodium species
Babesia microti
Clostridium perfingens
Bartonella bacilliformis

91
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Etiology
Which gene?

A

HFE gene defects

  1. C292Y mutations
  2. H63D mutations
92
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Is the absence of C282Y eliminate the diagnosis of hemochromatosis?

A

No

93
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Symptoms:

A

Fatigue, weakness, abdominal pain, arthralgia, mildly elevated liver enzymes;
Arthropathy (2nd and 3rd MTP joints);
DM, hypothyroidism, hypogonadism, depression, heart failure, and arrhythmia

94
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Advanced symptoms

A

Liver cirrhosis and liver failure

200 times increased the risk of liver cancer

95
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Whom should be screened?

A
  1. First-degree relatives with classic HFE-related hemochromatosis;
  2. Evidence of active liver disease;
  3. Abnormal iron study results obtained for other indications
96
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
What test should be used for screening?

A

Fasting serum transferrin saturation

>55%

97
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
What is the usage of ferritin level?

A

Supports diagnosis

Predict the development of symtpoms

98
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Who can be monitored

A

C282Y homozygous, normal serum ferritin level

99
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Who should be treated promptly

A

C282Y homozygous, elevated serum ferritin level

100
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Rx

A

Phlebotomy:
1 unit should be removed weekly
Ferritin level decreased to 10-5-ng/mL
Hematocrits maintained >30%

101
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
When to use iron chelation?

A

Phlebotomy is contraindicated.

Rarely used.

102
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
What special infections are they prone to get?

A

Vibrio vulnificus
Yersinia enterocolitica
Mucormycosis

103
Q

Iron overload syndromes
Primary/Hereditary hemochromatosis
Which kind food should they avoid?

A

Raw seafood

Undercooked pork

104
Q

Hemolytic anemia
Peripheral blood smear findings
Spherocytes

A

Hereditary spherocytosis

Autoimmune hemolytic anemia

105
Q

Hemolytic anemia
Peripheral blood smear findings
Target cell

A

Thalassemia
Hemoglobin C
Liver disease

106
Q

Hemolytic anemia
Peripheral blood smear findings
Schistocytes

A

Microangiopathic hemolytic anemia

107
Q

Hemolytic anemia
Peripheral blood smear findings
Bite cells

A

G6PD deficiency

108
Q

Hereditary spherocytosis

Supplement

A

Folic acid for moderate to severe HS

109
Q

Hereditary spherocytosis

Vaccination

A

Against encapsulated organisms:

Streptococcus pneumoniae; H. influenza and Neisseria meningitides before splenomegaly

110
Q

G6PD deficiency

A

Bite cells

Heinz bodies

111
Q

G6PD deficiency

Offending agents

A 
Rasburicase
Sulfa drugs
Fava beans
Some antimalaria medications
Infection
112
Q

Thalasemia

Why iron overload?

A

Because of ineffective erythropoiesis—>iron overload

113
Q

Thalasemia

Do you transfuse if anemic?

A

No!!!

Should rule out thalasemia otherwise worsening iron overload

114
Q

Sickle cell syndrome

What pain meds you CANNOT use?

A

Meperidine (lower seizure threshold)

115
Q

Sickle cell syndrome

What is ACS?

A

Acute chest syndrome

Vaso-occulsive involvement of the pulmonary vasculature

116
Q

Sickle cell syndrome

Dx of ACS?

A

New pulmonary infiltrates involving at least one complete lung segment that is consistent with alveolar consolidation

117
Q

Sickle cell syndrome

Sx of ACS

A

Chest pain, fever, tachypnea, wheezing or cough

118
Q

Sickle cell syndrome

DDx of ACS?

A

Infectious PNA
Fat embolissm
PE

119
Q

Sickle cell syndrome

What are the 3 main treatment strategies?

A

Preventive
Acute
Chronic

120
Q

Sickle cell syndrome

Mechanism?

A

Point mutation of beta-chain—>HbS

121
Q

Sickle cell syndrome

What is the treatment for pulmonary HTN

A

No treatment available right now

122
Q

Sickle cell syndrome
Ischemic stroke
What is the acute treatment?

A

Erythrocyte exchange transfuse

Aspirin

123
Q

Sickle cell syndrome
Ischemic stroke
What is the chronic treatment?

A

Chronic simple transfusion

Erythrocyte exchange transfuse (Target HbS<30-50%)

124
Q

Sickle cell syndrome
Ischemic stroke
Does HU instead of transufse for chronic treatment help

A

NO!

125
Q

Sickle cell syndrome
Ischemic stroke
What is the screening tool for children?

A

TranscranialDoppler Ultrasonography of the middle cerebral arteries (assess for increased velocity)

126
Q

Sickle cell syndrome
Hepatic crisis
Sx

A

Sudden onset RUQ pain
Liver enlargement
Acute anemia
Elevated liver enzymes

127
Q

Sickle cell syndrome
Hepatic crisis
Rx?

A

Transfuse

Erythrocyte exchange transfuse

128
Q

Sickle cell syndrome
CKD/Proteinuria
Preventive treatment?

A

Control BP<130/80

Secondary prevention: use ACEI or ARBs

129
Q

Sickle cell syndrome

When to transfuse?

A
  1. Significant anemia symptoms

2. End-organ damage

130
Q

Sickle cell syndrome

What is transfuse goal before surgery (low and media)

A

Hgb<10g/dL

131
Q

Sickle cell syndrome

What are the other preventive measure?

A

Vaccination

132
Q

What’s the difference of hemoglobin electropheresis between alpha-thalasemia and beta-thalasemia

A

Alpha-thalasemia: normal pattern

Beta-thalasemia: elevated HbA2 and HbF

Heme/Onco (19 decks)

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