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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
Iron deficiency anemia | How to test if there is GI malabsorpation of Iron supplement?
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
26
Iron deficiency anemia | Rx-How much oral iron need to give per day?
Total 150-200mg of iron divided by 2 or 3 times per day
27
Iron deficiency anemia | Rx- What is the indication of PRBC?
1. severe, symptomatic anemia 2. HD unstable patients 3. End-organ damage
28
Iron deficiency anemia | Rx- What are the side effects of oral supplement?
N/V, constipation, black stool, metallic taste
29
Iron deficiency anemia | DDx- When iron deficiency refractory to iron PO supplement?
1. Subclinical H.pylori infection-->treat infection | 2. Subclinical hypothyroidism-->give supplement of levothyroxine
30
Iron deficiency anemia | Rx-what is the indication for IV replacement?
1. GI malabsorption; 2. Inability to tolerate PO iron; 3. Poor response to oral iron; 4. Patients undergoing kidney dialysis receiving ESAs
31
Iron deficiency anemia | Rx- what are the formulation if IV iron?
1. Ferric gluconate: 12.5mg/mL 2. Iron dextran: 50mg/mL 3. Iron sucrose: 20mg/mL 4. Ferumoxytol: 30mg/mL
32
Inflammatory anemia | What it the labs characteristics?
Low TIBC <250; High ferritin >35; Low serum iron <60;
33
Anemia of kidney disease | What is the RBC morphology?
Normochromic; Normocytic; Can see burr cells
34
Anemia of kidney disease | What is the role of EPO level in dx?
NOT useful
35
Vitamin B12 deficiency | What are 2 other chemicals increased?
1. Methylmalonic acid (MMA); | 2. Homocystein levels.
36
Vitamin B12 deficiency | What is the morphology change of blood cells?
1. Macrocytosis; 2. Hemolysis of erythrocytes within the bone marrow; 3. Hypersegmentation in granulocytes
37
Vitamin B12 deficiency | What are causes?
1. Gastric,bariatric, or ileal surgery; 2. IBD; 3. pernicious anemia
38
Vitamin B12 deficiency | How long dose it take to be deficient?
2-3 years
39
Vitamin B12 deficiency | Clinical manifestation
``` Anemia; Hemolysis; Neurologic dysfunction: numbness, decreased vibratory sense, gait problems, neuropsychiatric symptoms (can present without anemia) Glossitis; Hyperpigmentation; Infertility ```
40
Vitamin B12 deficiency | Pernicious anemia, what other comorbidities?
Vitiligo; DM; Thyroid disease.
41
Vitamin B12 deficiency | What peripheral blood smear show?
Large oval erythrocytes; Hypersegmented neutrophils (>6 lobes); Pancytopenia.
42
Vitamin B12 deficiency | Dx-first step?
Serum cobalamin level
43
Vitamin B12 deficiency Dx What is the serum cobalamin level indicating unlikely deficiency?
>300pg/mL (221 pmol/L)
44
Vitamin B12 deficiency Dx What is the serum cobalamin level indicating very likely deficieny?
<200pg/mL
45
Vitamin B12 deficiency | What is the senstivity and sepicificity of measuring Cobalamin level
very poor
46
Vitamin B12 deficiency | When should level be checked?
Before starting treatment
47
Vitamin B12 deficiency Dx- What else to measure?
MMA and Homocysteine
48
Vitamin B12 deficiency Dx- What level of MMA indicating deficiency?
MMA>500nmol/L | can increase in AKI
49
Vitamin B12 deficiency Dx- Is bone marrow biopsy needed?
No | It is confusing. Only when not responding
50
Vitamin B12 deficiency Dx- When is paritel cell antibody elevated?
1. Pernicious anemia | 2. Chronic gastritis
51
Vitamin B12 deficiency Dx- Pernicious anemia
Intrinsic factor antibody
52
Vitamin B12 deficiency Rx: Initiating step
High-dose PO 1000-2000 ug/d even in intrinsic factor insufficiency
53
Vitamin B12 deficiency Rx: When to start IM or IV supplement?
1. Severe anemia; 2. Neurologic dysfunction; 3. Not responding to PO replacement
54
Vitamin B12 deficiency Rx IM management
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
Vitamin B12 deficiency F/u When to check Reti count?
1 week
56
Vitamin B12 deficiency | Who is at risk?
Vegetarians
57
Folate Deficiency | Who are at risk?
Older adults, nursing homes, alcoholics
58
Folate Deficiency | Medication causing folate deficiency?
Phenytoin; Trimethoprim; MTX
59
Folate Deficiency | Diseases causing deficiency?
IBD, short gut syndrome, celiac disease
60
Folate Deficiency | High demand causing deficiency?
Pregnancy; Lactation; States of chronic hemolysis; Exfoliant dermatitis
61
Folate Deficiency | Dx
Folate; (good screening tool, can fluctuate with meals) High homocysteine MMA wnl
62
Folate Deficiency | Rx
PO folic acid | 5mg daily until complete hemotologic recovery
63
Folate Deficiency Rx Is PO supplement suffcient in malabsorption?
Yes
64
Folate Deficiency | What need to r/o before Rx?
B12 deficiency | Because it will make them more vulnerable to central and peripheral nervous symptoms
65
Thalassemia | Normal Hemoglobin
Hemoglobin A (alpha2beta2)
66
Thalassemia | Hemoblogin A2
alpha2delta2
67
Thalassemia | Hemoblogin F
alpha2gama2
68
Thalassemia | alpha-thalassemia
Duplication of the alpha-blobin chain on chromosome 16 results 4 alph-globins genes (aa/aa)
69
Thalassemia alpha-thalassemia If absence of 1 alpha gene Sx:
(-a/aa) | Asymptomatic carrier state
70
Thalassemia alpha-thalassemia If absence of 2 alpha gene Sx
(--/aa) (-a/-a) | Mild microcytic anemia
71
Thalassemia alpha-thalassemia If absence of 3 alpha gene Sx:
(--/-a) Hemoglobin H disease | Hemolysis, splenomegaly
72
Thalassemia alpha-thalassemia If absence of 4 alpha gene Sx:
Hydrops fetalis and intrauterine fetal demise
73
Thalassemia Beta-thalassemia Categories
Thalaseemia minor, intermedia, and major
74
Thalassemia Beta-thalassemia Minor mutations:
Heterozygous gene mutation
75
Thalassemia Beta-thalassemia Minor Sx
Asymptomatic, microcytic mild anemia
76
Thalassemia Beta-thalassemia Intermedia Sx
Moderate hemolytic anemia, Hgb>7 without transfusion support; Worsening anemai if aplastic crisis during infection
77
Thalassemia Beta-thalassemia Intermedia Complications
Chronic hemolysis, folate deficiency, chlelithiasis
78
Thalassemia Beta-thalassemia Major Sx:
Early life with pallor, failure to thrive, severe hemolytic anemia, erythroid hyperplasia in the bone marrow, bone defomities, massive HSM, extramedullary hematopoiesis
79
Thalassemia Beta-thalassemia Rx:
1. Transfusion if Hgb<7 | 2. Transplant can be curative.
80
Thalassemia Beta-thalassemia Complications
Iron overload -->caridomyopathy, liver fibrosis, endocrinology problem;
81
Thalassemia Beta-thalassemia Rx of iron overload
SQ desferrioxamine or PO deferasirox, deferiprone
82
Sickle cell anemia | Rx strategies
1. Transplant 2. Prophylatic transfusion 3. Hydroxyurea
83
Sickle cell anemia | When to give hydroxyurea
>=1-2 episodes of vaso-occlusive episodes
84
Hemolytic anemia | Other causes of hemolysis
Macroangiopathic hemolytic anemia March hemoglobinuria Hemolysis associated with chemicals and physical agents Hemolysis from infections
85
Hemolytic anemia Macroangiopathic hemolytic anemia Etiology
Heart valves (mechanical heart valve) Intracardiac tumors Rupture of chordae tendinea
86
Hemolytic anemia Macroangiopathic hemolytic anemia Lab findings
Schistocytes
87
Hemolytic anemia March Hemoglobinuria Etiology
Repetitive trauma to soles or palms, seen in marching, ranning, drum playing, karate or head baning
88
Hemolytic anemia March hemoglobinuria Lab findings
Positive urine hemosiderin test
89
Hemolytic anemia | Hemolysis associated with chemical and physical agents
Arsenic Elevated copper (Wilson disease) Insect and spider bites (Brown recluse spider) Severe burns (direct thermal injury to RBCs)
90
Hemolytic anemia Hemolysis from infections Causes
Plasmodium species Babesia microti Clostridium perfingens Bartonella bacilliformis
91
Iron overload syndromes Primary/Hereditary hemochromatosis Etiology Which gene?
HFE gene defects 1. C292Y mutations 2. H63D mutations
92
Iron overload syndromes Primary/Hereditary hemochromatosis Is the absence of C282Y eliminate the diagnosis of hemochromatosis?
No
93
Iron overload syndromes Primary/Hereditary hemochromatosis Symptoms:
Fatigue, weakness, abdominal pain, arthralgia, mildly elevated liver enzymes; Arthropathy (2nd and 3rd MTP joints); DM, hypothyroidism, hypogonadism, depression, heart failure, and arrhythmia
94
Iron overload syndromes Primary/Hereditary hemochromatosis Advanced symptoms
Liver cirrhosis and liver failure | 200 times increased the risk of liver cancer
95
Iron overload syndromes Primary/Hereditary hemochromatosis Whom should be screened?
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
Iron overload syndromes Primary/Hereditary hemochromatosis What test should be used for screening?
Fasting serum transferrin saturation | >55%
97
Iron overload syndromes Primary/Hereditary hemochromatosis What is the usage of ferritin level?
Supports diagnosis | Predict the development of symtpoms
98
Iron overload syndromes Primary/Hereditary hemochromatosis Who can be monitored
C282Y homozygous, normal serum ferritin level
99
Iron overload syndromes Primary/Hereditary hemochromatosis Who should be treated promptly
C282Y homozygous, elevated serum ferritin level
100
Iron overload syndromes Primary/Hereditary hemochromatosis Rx
Phlebotomy: 1 unit should be removed weekly Ferritin level decreased to 10-5-ng/mL Hematocrits maintained >30%
101
Iron overload syndromes Primary/Hereditary hemochromatosis When to use iron chelation?
Phlebotomy is contraindicated. | Rarely used.
102
Iron overload syndromes Primary/Hereditary hemochromatosis What special infections are they prone to get?
Vibrio vulnificus Yersinia enterocolitica Mucormycosis
103
Iron overload syndromes Primary/Hereditary hemochromatosis Which kind food should they avoid?
Raw seafood | Undercooked pork
104
Hemolytic anemia Peripheral blood smear findings Spherocytes
Hereditary spherocytosis | Autoimmune hemolytic anemia
105
Hemolytic anemia Peripheral blood smear findings Target cell
Thalassemia Hemoglobin C Liver disease
106
Hemolytic anemia Peripheral blood smear findings Schistocytes
Microangiopathic hemolytic anemia
107
Hemolytic anemia Peripheral blood smear findings Bite cells
G6PD deficiency
108
Hereditary spherocytosis | Supplement
Folic acid for moderate to severe HS
109
Hereditary spherocytosis | Vaccination
Against encapsulated organisms: | Streptococcus pneumoniae; H. influenza and Neisseria meningitides before splenomegaly
110
G6PD deficiency
Bite cells | Heinz bodies
111
G6PD deficiency | Offending agents
``` Rasburicase Sulfa drugs Fava beans Some antimalaria medications Infection ```
112
Thalasemia | Why iron overload?
Because of ineffective erythropoiesis—>iron overload
113
Thalasemia | Do you transfuse if anemic?
No!!! | Should rule out thalasemia otherwise worsening iron overload
114
Sickle cell syndrome | What pain meds you CANNOT use?
Meperidine (lower seizure threshold)
115
Sickle cell syndrome | What is ACS?
Acute chest syndrome | Vaso-occulsive involvement of the pulmonary vasculature
116
Sickle cell syndrome | Dx of ACS?
New pulmonary infiltrates involving at least one complete lung segment that is consistent with alveolar consolidation
117
Sickle cell syndrome | Sx of ACS
Chest pain, fever, tachypnea, wheezing or cough
118
Sickle cell syndrome | DDx of ACS?
Infectious PNA Fat embolissm PE
119
Sickle cell syndrome | What are the 3 main treatment strategies?
Preventive Acute Chronic
120
Sickle cell syndrome | Mechanism?
Point mutation of beta-chain—>HbS
121
Sickle cell syndrome | What is the treatment for pulmonary HTN
No treatment available right now
122
Sickle cell syndrome Ischemic stroke What is the acute treatment?
Erythrocyte exchange transfuse | Aspirin
123
Sickle cell syndrome Ischemic stroke What is the chronic treatment?
Chronic simple transfusion | Erythrocyte exchange transfuse (Target HbS<30-50%)
124
Sickle cell syndrome Ischemic stroke Does HU instead of transufse for chronic treatment help
NO!
125
Sickle cell syndrome Ischemic stroke What is the screening tool for children?
TranscranialDoppler Ultrasonography of the middle cerebral arteries (assess for increased velocity)
126
Sickle cell syndrome Hepatic crisis Sx
Sudden onset RUQ pain Liver enlargement Acute anemia Elevated liver enzymes
127
Sickle cell syndrome Hepatic crisis Rx?
Transfuse | Erythrocyte exchange transfuse
128
Sickle cell syndrome CKD/Proteinuria Preventive treatment?
Control BP<130/80 | Secondary prevention: use ACEI or ARBs
129
Sickle cell syndrome | When to transfuse?
1. Significant anemia symptoms | 2. End-organ damage
130
Sickle cell syndrome | What is transfuse goal before surgery (low and media)
Hgb<10g/dL
131
Sickle cell syndrome | What are the other preventive measure?
Vaccination
132
What’s the difference of hemoglobin electropheresis between alpha-thalasemia and beta-thalasemia
Alpha-thalasemia: normal pattern | Beta-thalasemia: elevated HbA2 and HbF

Heme/Onco (19 decks)

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