Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Clinical Medicine 1 > Hematology 1- Iron, Thalassemia, Chronic Dz, Sideroblastic, Lead 10/21/16 > Flashcards

Hematology 1- Iron, Thalassemia, Chronic Dz, Sideroblastic, Lead 10/21/16 Flashcards

1
Q

What is Ferritin?

A

Storage form of iron. Easily accessible and small.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is Transferrin?

A

Iron transport protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is TIBC? What does it indirectly measure?

A

Total iron binding capacity. Measure of iron binding capacity (and indirect assessment of transferrin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is SI (Serum Iron)?

A

Amount of transferrin saturated of Iron Fe2+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is % saturation and formula formula?

A

transferrin saturation SI/TIBC x 100

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is Reticulocyte Index? (RI)

A

Direct measure of bone marrow function. If keeping up with RBC need.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is Hemosiderin? Can the body use it easily?

A

Complex iron stores in macrophages. Difficult for the body to use.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

HCT normal range?

A

36-45

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hgb normal range?

A

12-15

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

WBC normal range?

A

5000-10,000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Platelet normal range?

A

150,000-400,000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Normal body iron range?

A

3-4g

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Define Anisocytosis. Which lab value?

A

Different size RBCs. Low MCV.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Define Poiklocytosis

A

Different shaped cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

MCV normal range?

A

80-100

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MCHC normal range?

A

33-35

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Hypochromic from which value?

A

MCHC lower than 33.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Average American iron intake?

A

10-15mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Percent of dietary iron absorbed?

A

10%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Iron requirement for male and non-mensturating females?

A

1mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Iron requirement for mensturating females range?

A

3-4mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Iron intake for pregger female range?

A

2-5mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Normal body iron content range?

A

3-4g

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How much iron lost through daily exfoliation in gut?

A

1mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

How much iron loss due to menses? Menses length range?

A

1mg/day, x4-7 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Entire iron loss from preggers, delivery, and nursing?

A

About 1000mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

How much Fe lost in bleeding per 100mL of whole blood? (Trauma, acute bleed, loss d/t surgery, etc)

A

50mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

How much Fe in 100mL of whole blood?

A

50mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Who gets Iron Deficiency Anemia most often?

A

Women more than men. D/t mensturation and preggers.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is #1 cause of Iron Deficiency Anemia until proven otherwise?

A

BLEEDING!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Iron Deficiency Anemia causes NIMBLE stands for?

A 
N=Need
I=Intake
M=Malabsorption
B=Blood
L=Loss
E=Excessive donation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Iron Deficiency Anemia causes other than bleeding?

A
  1. Deficient diet
  2. Decreased absorption (Celiac dz, Zn deficiency)
  3. Increased requirements (preggers, lactation)
  4. Hemoglobinuria
  5. Iron sequestration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

3 stages of Iron Deficiency Anemia?

A
  1. Depletion of iron stores without anemia
  2. Anemia with normal RBC size (normal MCV)
  3. Anemia with reduced RBC size (low MCV)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Signs and symptoms of Iron Deficiency Anemia?

A

Fatigue, dizzy/weak, HA, tachycardia, dyspnea on exertion, palpitations

Beeturia, pica (eating weird things), pagophagia (craving for ice), dysphagia d/t esophageal web), pallor, conjunctival and palm pallor, smooth tongue, brittle nails, kolionychia (nails spooning), cheiolosis (breakdown of skin in corner of mouth when severe)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Three signs/symptoms of severe anemia? Why?

A

Tachycardia, dyspnea on exertion, palpitations. Due to decreased oxygen carrying capacity.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

In Iron Deficiency labs (Serum Ferritin, Serum Iron, TIBC, and % Sat) which are low and increased?

A

Serum Ferritin=low
Serum Iron=low
% Sat=low
TIBC=increased

MCV low in anemia w/reduced RBC size.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

MCV and MCHC in initial Iron Deficiency Anemia?

A

Low MCV, low MCHC. Microcytic, hypochromic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

MCV and MCHC in severe Iron Deficiency Anemia? Aniso or Poik?

A

Low MCV, low MCHC.
Progression to anisocytosis and poikilocytosis.
Aniso=target cells
Poik=pencil and cigar shapes cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Essentials of Dx for Iron Deficiency Anemia

A

Iron deficiency present.
Serum Ferritin less than 12 in absence of scurvy
-OR-
Anemia and Serum Ferritin less than 30

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

How to prevent Iron Deficiency Anemia in pregnant women in deficient?

A

Low dose Iron 30mg/day PO if deficient

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

When to screen girls 12-18 and nonpreggers childbearing age women for Iron Deficiency Anemia?

A

Screen q5-10y, annual screening for women at risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

In Iron Deficiency Anemia a Serum Ferritin less than __ and in the absence of ______

A

Serum Ferritin less than 12 in absence of scurvy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

In Iron Deficiency Anemia a PT with anemia and a Serum Ferritin less than __ has essential to dx?

A

Anemia and Serum Ferritin less than 30

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is Reticulocyte Index value in Iron Deficiency Anemia?

A

Normal at less than 2.5.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What is #1 think to do for treating Iron Deficiency Anemia?

A

Search for blood loss! Do stool guaiac or colonoscopy to check for occult blood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What is the DOC for iron repletion in Iron Deficiency Anemia?

A

Ferrous Sulfate 325mg (65mg elemental iron) PO TID x1-2 months, then daily for 3-6 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What will CBC be at 3 weeks, 2 months, and 6 months after starting tx for Iron Deficiency Anemia?

A

3 weeks=half way to normal
2 months=normal
6 months=normal (replace storage)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

In Iron Deficiency Anemia if CBC is normal at 2 months why continue tx for 6 months?

A

To replete iron stores

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is #1 ADR or PO Iron?

A

Constipation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

How often to check for Iron Deficiency Anemia after tx completed?

A

Once a year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What to avoid when taking PO Iron?

A

Avoid dairy, fiber, food, tea, tannins, phosphates, antacids. All bind iron and prevent absorption.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What helps PO Iron absorption?

A

Orange Juice! Drink OJ!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

When to use IV iron for Iron Deficiency Anemia?

A

If they fail PO iron. Might have GI malabsorption from IBD or gastric bypass or might have continued blood loss that can’t be stopped (hemodialysis).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

If PT on dialysis and not being transfused regularly and has Iron Deficiency Anemia what to do?

A

Supplement with IV Iron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

If PT on dialysis and is being transfused regularly and has Iron Deficiency Anemia what to do?

A

Don’t supplement iron!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

When and how to take PO Iron?

A

Empty stomach. Use straw to avoid teeth stain.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

If PO Iron not tolerated BID or TID what to do?

A

Use low dose at 1-2x/day or slow absorption prep (SlowFe)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Most common ADRs of PO Iron?

A

Nausea, constipation, diarrhea, dark stool

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

If PT not responding to PO Iron for Iron Deficiency Anemia what to consider?

A

Incorrect dx, celiac dz, ongoing bleeding, non-compliance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

When to refer Iron Deficiency Anemia to hematology

A

If H and H below 9 and below 27%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What are signs of Zinc Deficiency on hands and buttocks?

A

Rashes.
Buttocks=dry, scaly, eczematous skin. Can become infected with Candida albicans.
Hands=enlarged fingers, paronychia, bright erythema on terminal phalanges

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What can Zinc Deficiency cause malabsorption of?

A

Iron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Thalassemia is a reduction of what? What does it do to hemoglobin synthesis?

A

Reduction in synthesis of alpha or beta globin chains. Causes reduced hemoglobin synthesis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Is Thalassemia hereditary?

A

YES!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

What happens to MCV and MCH in Thalassemia?

A

Low MCV, low MCH. Hypochromic and microcytic anemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Heme is made up of how many porphyrin and rings and iron molecules?

A

Heme=4 porphyrin rings, 4 iron molecules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What is normal adult Globin made up of?

A

HgA. 2 alpha and 2 betas chains.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Alpha globin on which chromosome, how many sites, and how many pairs? Total from parents?

A

Chromosome 16, 4 sites, 2 from each parent. 4 total.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Beta globin on which chromosome and how many pairs? Total from parents?

A

Chromosome 11, 2 sites, 1 from each parent.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Beta globin chains can be mimicked by what?

A

Delta chains or Gamma chains.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Can Alpha globin chains be mimicked?

A

No.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Hgb A normally made up of which globin chains? How many? What percent of normal adult circulation

A

2 alpha, 2 beta.

98% of normal adult circulation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Fetal hemoglobin is called what, made up of which globin chains, and does what to oxygen? Wears off when?

A

Hgb F. 2 alpha, 2 gamma.

Very high affinity to oxygen, steals oxygen from mother. Wears off around 6 months.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Hgb H is made up of which and how many globin chains?

A

4 Beta chains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

3 types of Thalassemia and transfusions?

A
  1. Trait= lab findings, no clinical impact
  2. Intermedia= moderate clinical impact, occasional transfusion
  3. Major= Life threatening, transfusion dependent
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Thalassemia iron study lab trends (SF, SI, TIBC, % Sat)?

A

SF=norm or inc
SI=norm or inc
% Sat=norm or ince
TIBC=NORMAL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Thalassemia RBC, MCV, MCH, Reticulocyte Count?

A

RBC=norm or elevated
MCV=LOW (lower than iron deficinecy)
MCH=low
Reticulocyte Count=elevated, above 2.5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

What are Heinz Bodies (found in Thalassemia)?

A

Denatured hemoglobin found inside RBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Are Nucleated RBCs (NRBCs) found with Thalassemia?

A

Yup, you bechya.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Thalassemia essentials of Dx

A
  1. Microcytosis disproportionate to degree of anemia
    • fam history
  3. Norm or elevated RBC count
  4. Abnormal RBC morphology with microcytes, hypochromia, acanthocytes, and target cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

What are acanthocytes?

A

Spiked/spurred RBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Which 2 hemoglobins elevated in Beta-Thalassemia?

A
  1. Hgb A2

2. Hgb F

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

Alpha-Thalassemia epi?

A

Southeast Asia and China

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Genetic eti of Alpha-Thalassemia?

A

Gene deletions from Chromosome 16.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Each Alpha-Thalassemia gene deletion causes reduced alpha-globin chain synthesis by how much?

A

1/4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

If missing two Alpha-Thalassemia sites on Chromosome 16 how much alpha-globin reduced by?

A

50%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

Can alpha chains be mimicked or replaced by other chains?

A

No! Beta chains can, but alpha aren’t beta chains.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

What is the genotype of normal and silent carrier Alpha-Thalassemia?

A

Normal= aa/aa

Silent carrier= aa/a-

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

What are the MCV and hematocrit or normal and silent carrier Alpha-Thalassemia?

A

Normal MCV, normal crit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

What are genotypes for Alpha-Thalassemia Minor?

A

aa/– or a-/a-

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

What are MCV and hematocrit for Alpha-Thalassemia Minor

A

MCV=60-75

Crit=28-40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

What is genotype for Alpha-Thalassemia Hemoglobin H Dz?

93
Q

What are MCV and hematocrit for Alpha-Thalassemia Minor Hemoglobin H Dz?

A

MCV=60-75

Crit=22-32%

94
Q

What is genotype for Alpha-Thalassemia Hydrops Fetalis?

A

MCV=less than 60

Crit=less than 18%

95
Q

In Alpha-Thalassemia-1-Trait what is genetic eti and epi? Heterozygous or homozygous?

A

Alpha deletion heterozygous. a-/a-, each parent missing one site from Chromomsome 16.
Asian descent.

96
Q

In Alpha-Thalassemia-2-Trait what is genetic eti and epi? Heterozygous or homozygous?

A

Alpha deletion homozugous. aa/–, one parent missing two sites from Chromomsome 16.
Black descent.

97
Q

Life expectancy of Alpha-Thalassemia-Trait? Treatment?

A

Normal life expectancy. No treatment, just need to let them know.

98
Q

What severity of anemia does Alpha-Thalassemia-Trait have?

A

Mild microcytic anemia.

99
Q

What is the genetic eti of Alpha Thalassemia – Hgb H Disease? (Hint: How many sites missing?)

A

a-/–. 3 sites missing. Alpha deletion is heterozygous.

100
Q

What type of anemia in Alpha Thalassemia – Hgb H Disease?

A

Chronic hemolytic anemia

101
Q

What is the variability of severity in Alpha Thalassemia – Hgb H Disease?

A

Variable from Thalassemia minor to Thalassemia intermedia.

102
Q

What are the signs and symptoms of Alpha Thalassemia – Hgb H Disease? PE findings?

A

Fatigue and weakness.

PE: Pallor and splenomegaly

103
Q

What severity of anemia in Alpha Thalassemia – Hgb H Disease?

A

Moderate anemia. HCT 22-32 (36-45 norm).

104
Q

RBC in Alpha Thalassemia – Hgb H Disease?

A

Normal or increased

105
Q

Reticulocyte Count in Alpha Thalassemia – Hgb H Disease?

106
Q

Does does Peripheral Smear show for Alpha Thalassemia – Hgb H Disease?

A

Markedly abnormal, microcytes, target cells, poikliocytosis

107
Q

What does hemoglobin electrophoresis show on Alpha Thalassemia – Hgb H Disease?

A

10-40% Hgb H

108
Q

What is MCV in Alpha Thalassemia – Hgb H Disease?

A

Low at 60-70. (80-100 norm)

109
Q

Tx for Alpha Thalassemia – Hgb H Disease?

A

Folic Acid 1mg PO daily. Usually not transfusion dependent.

110
Q

What to avoid with Alpha Thalassemia – Hgb H Disease?

A

Avoid medicinal iron and oxidative drugs like Sulfonamides which destabilize hemoglobin.

111
Q

Tx if iron overloaded?

A

Iron chelation

112
Q

Is Alpha Thalassemia – Hgb H Disease transfusion dependent? When?

A

Usually not. Only for hemolytic exacerbation like infx or stress or aplastic crisis.

113
Q

Where is iron in Alpha Thalassemia – Hgb H Disease?

A

Still in system but not bound and becomes overloaded.

114
Q

What is genetic eti of Alpha-thalassemia - Hydrops Fetalis?

A

–/–. All 4 sites missing. Alpha deletion heterozygous.

115
Q

How much alpha globin is made in Alpha-thalassemia - Hydrops Fetalis?

116
Q

What happens to baby with Alpha-thalassemia - Hydrops Fetalis?

A

Stillborn. Incompatible with extra-uterine life.

117
Q

What is HCT and MCV os Alpha-thalassemia - Hydrops Fetalis?

A

Hct=less than 18% (36-45)

MCV=less than 60 (80-100)

118
Q

Tx for Alpha-thalassemia - Hydrops Fetalis?

A

Fetal diagnosis with transfusion and/or parental stem cell infusion followed by allogenic bone marrow transplant has led to some survival.

119
Q

Epi of Beta-thalassemia?

A

Major Mediterranean origin (Italian, Greek). Lesser Asian and Black population.

120
Q

What is reduced in Beta-thalassemia?

A

Reduced Beta-globin chain synthesis.

121
Q

Which chromosome is Beta globin gene located on? How many?

A

2 genes, 1 from each parent. Chromosome 11.

122
Q

Can Beta globin be mimicked?

A

YES, by gamma and delta chains.

123
Q

What is the type genetic mutation in Beta-thalassemia?

A

Point gene mutation, not gene deletion.

124
Q

Children with Beta-thalassemia look like what until 6 months?

A

Normal at first, severe anemia after 6 months d/t Hgb F ceasing production and needing Beta chains.

125
Q

In Beta-thalassemia what does Beta+ mean?

A

Reduced (not absent) synthesis of beta chains

126
Q

In Beta-thalassemia what does Beta0 mean?

A

Absent beta chain expression

127
Q

What are the two Beta-like globins?

A

Gamma and Delta

128
Q

HemF and oxygen delivery to tissues?

A

HemF has high affinity for oxygen and doesn’t easily release oxygen to tissues

129
Q

What are the two types of Hgb in Beta-thalassemia?

A

Hgb A2 or Hgb F

130
Q

Beta-thalassemia and RBC morphology?

A

Abnormal RBC morphology w/microcytes, hypochromia, acanthocytes

131
Q

Beta-thalassemia should be considered if….?

A

Low MCV, normal ferritin, family hx

132
Q

What is genetic eti of Beta-thalassemia-Minor?

A

Beta/Beta+ or Beta/beta0. Heterozygous.

133
Q

What degree of anemia in Beta-thalassemia-Minor?

A

Clinically insignificant microcytic anemia

134
Q

MCV in Beta-thalassemia-Minor?

A

Low 55-75 (80-100)

135
Q

Hct in Beta-thalassemia-Minor?

A

Modest anemia 28-40 (36-45)

136
Q

RBC in Beta-thalassemia-Minor?

A

RBC=Normal or increased

137
Q

Reticulocyte Count in Beta-thalassemia-Minor?

A

Normal to slight increase (but poor product)

138
Q

Peripheral smear in Beta-thalassemia-Minor?

A

Mild abnormalities: microcytes, target cells, basophillic stippling

139
Q

What does Hemoglobin electrophoresis show in Beta-thalassemia-Minor?

A

Elevated Hgb A2 at 4-8%. Occasional Hgb F at 1-5%.

140
Q

Tx for Beta-thalassemia-Minor?

A

No transfusions or tx needed. Identify so they don’t go through repeat blood tests over and over. Refer to genetic counseling.

141
Q

Genetic eti of Beta-thalassemia-Intermedia?

A

Beta+/Beta+. Homozygous but allows for higher rate of beta globin synthesis.

142
Q

PE of Beta-thalassemia-Intermedia?

A
  • Bony deformity d/t RBC production outside bone marrow
  • -Facial abnormality, pathological fx
  • Hepatosplenomegaly
  • Norm at birth, severe anemia at 6months
143
Q

HCT in Beta-thalassemia-Intermedia?

A

17-33% (36-45)

144
Q

MCV in Beta-thalassemia-Intermedia?

A

Low 55-75 (80-100)

145
Q

Reticulocyte Count in Beta-thalassemia-Intermedia?

146
Q

RBC morphology on peripheral smear in Beta-thalassemia-Intermedia?

A

Hypochromia, microcytosis, basophillic stippling, target cells

147
Q

Hemoglobin electrophoresis in Beta-thalassemia-Intermedia?

A

Hgb A up to 30%, Hgb A2 up to 10%, HgbF 6-100%

148
Q

Tx for Beta-thalassemia-Intermedia?

A

Regular transfusions not necessary, only occasionally. Refer to genetic counseling.

149
Q

Survival for Beta-thalassemia-Intermedia?

A

Survive in adulthood and do well.

150
Q

What type of anemia in Beta-thalassemia-Intermedia?

A

Chronic hemolytic anemia

151
Q

Beta-thalassemia-Major aka?

A

Cooley anemia

152
Q

Beta-thalassemia-Major genetic eti?

A

Beta0/Beta0 or Beta+/Beta+. Homozygous. Two abnormal sites or no sites at all.

153
Q

When is Beta-thalassemia-Major diagnosed?

A

6-12 months of age

154
Q

Beta-thalassemia-Major birth to 6 months?

A

Normal at birth, severe anemia at 6 months.

155
Q

What type of RBC production in Beta-thalassemia-Major?

A

Extramedullary Hematopoiesis=RBCs being made outside bone marrow

156
Q

PE of Beta-thalassemia-Major?

A
  • Stunted growth, bone deformity (face deformed, pathologic fx)
  • Hepatosplenomegaly
  • Jaundice w/gallstones or hepatitis related cirrhosis
  • Thrombophillia
157
Q

Other complications with Beta-thalassemia-Major

A

So many…..liver, lungs, bones, heart, diabetes, etc

158
Q

RBC and indicies with Beta-thalassemia-Major?

A

High red count, low indicies, large amount of odd looking cells

159
Q

HCT and MCV in Beta-thalassemia-Major?

A

HCT=less than 10 (36-45) severe anemia

MCV=LOW

160
Q

Peripheral smear with Beta-thalassemia-Major?

A

Bizarre looking cells, severe poikilocytosis (diff shaped cells), hypochromia, microcytosis, target cells, basophillic sippling, NRBC

161
Q

Hemoglobin electrophoresis with Beta-thalassemia-Major?

A

Little or no HgbA, variable Hgb A2, predominant Hgb F

162
Q

TOC for Beta-thalassemia-Major?

A

-TOC=Allogenic stem cell transplant (only cure available)

163
Q

Blood transfusions and other tx for Beta-thalassemia-Major?

A

Yes. Transfuse PRBC to prevent endogenous hematopoiesis.

  • Maintain Hgb 9-10
  • Iron Chelation if regular transfusions
  • Folic Acid 1mg PO daily
  • Splenectomy if indicated
164
Q

When considering Thalassemia which tests to start with?

A

Start with CBC. If MCV/MCH low than consider Thalassemia.

165
Q

Retic count is low in which type of Thalassemia?

A

Beta-Thalassemia-Major

166
Q

HgbF at 90-100% in which type of Thalassemia?

A

Beta-Thalassemia-Major

167
Q

Bone deformities in homozygous Beta-Thalassemia due to what?

A

Extramedullary hematopoiesis

168
Q

Essentials of Thalassemia diagnosis?

A

-Microcytosis disproportionate to degree of anemia
-Positive family history or lifelong personal history of microcytic anemia (Low MCV)
-Normal or elevated RBC count
-Abnormal RBC morphology with microcytes, hypochromia, acanthocytes,
and target cells

169
Q

Complications of chronic transfusions?

A

Transfusional hemosiderosis=iron overload. Builds up in liver and heart. Chelate!

170
Q

When to refer Thalassemia?

A
  • Severe to hematologist
  • Minor or intermediate to genetic counselor
  • Any PT with unexplained microcytic anemia (MCV less than 80)
171
Q

What is the “Vampire” disease?

172
Q

Porphyria eti?

A

Altered activities of enzymes within heme biosynthetic pathway. Can’t produce porphyrin ring.

173
Q

Porphyria signs and symptoms?

A

Anemia, ver pale skin, photosensitive (blisters in sun), dental abnormalities (pointed teeth)

174
Q

Anemia of Chronic Dz labs? (Hint: 5)

A

CBC=mild anemia, normochromic, normoctyic
RI=low to normal, under 2.5
ESR and CRP=elevated
Iron Studies:

SF=norm or inc
SI=low (inflammation), normal (organ failure and elderly)
TIBC=norm or low
% Sat=norm or low

175
Q

Gold standard for Anemia of Chronic Dz? How often done?

A

Bone marrow biopsy w/iron staining. Rarely done!

176
Q

Anemia of Chronic Dz signs and symptoms

A
  • Hx of known chronic dz, exam findings related to it

- Sx of typical anemla: fatigue, dizziness, weakness

177
Q

3 Types of Anemia of Chronic Dz?

A
  1. Anemia of inflammation
  2. Anemia of Organ Failure
  3. Anemia of Elderly
178
Q

Anemia of Inflammation epi?

A

People with known chronic dz

179
Q

Anemia of Inflammation iron etiology?

A

Reduced uptake of iron from gut into bone marrow. Reduced response to EPO.

180
Q

What happens to RBCs in Anemia of Inflammation?

A

Shortened RBC survival and hemolytic (breakdown) of RBCs

181
Q

What is the “inflammatory marker” in Anemia of Inflammation?

182
Q

Anemia of Inflammation seen in PTs who have what diseases?

A

Inflammatory disorder, infectious process, or malignancy.

EX: IBD, RA, chronic infection, malignancy, severe trauma, critically ill patients

183
Q

What do cytokines to in Anemia of Inflammation?

A

Decrease RBC production

184
Q

Anemia of Inflammation workup?

A

CBC, RI, Iron Studies

185
Q

What sort of anemia seem in Anemia of Inflammation?

A

Normocytic or microcytic anemia

186
Q

Anemia of Inflammation and ESR and CRP?

187
Q

Which iron type can be high in acute phase of Anemia of Inflammation?

188
Q

PTs with Anemia of Inflammation should be evaluted for which two possible coexistent issues?

A

Iron deficiency

Folic acid deficiency

189
Q

What is SI in Anemia of Inflammation?

190
Q

Anemia of Elderly is what type of diagnosis?

A

Dx of exclusion

191
Q

Anemia of Elderly eti? (Hint: EPO and inflammation)

A
  • Resistance to EPO

- Decrease to EPO production, d/t low level infammation

192
Q

Anemia of Elderly has underlying issue of what?

A

Underlying chronic dz

193
Q

Anemia of Elderly serum iron levels?

194
Q

Anemia of Elderly ESR or CRP levels?

195
Q

Anemia of Elderly presentation?

A

Fatigue, dizzy, weak. Sx and exam findings related to chronic dz.

196
Q

Anemia of Organ Failure EPO and RBC mass?

A
  • Reduced EPO production

- RBC mass decreased

197
Q

Anemia of Organ Failure associated with what?

A

Chronic inflammation

198
Q

Anemia or Organ Failure can coexist with what?

A

Iron deficiency or folic acid deficiency

199
Q

ESR and CRP are specific or non-specific inflammatory markers?

A

Non-specific

200
Q

Serum Ferritin below what indicates coexistent iron deficiency?

A

Less than < 30ng/mL indicates coexistent iron deficiency

201
Q

Low Serum Iron (SI) associated with which Anemia of Chronic Dz?

A

Inflammatory

202
Q

Normal Serum Iron (SI) associated with which Anemia of Chronic Dz?

A

Organ failure and elderly

203
Q

Tx for Anemia of Chronic Dz?

A
  • PRBC if indicated
  • Treat underlying dz
  • Treat folic acid of iron deficiency
  • Parenteral recombinant EPO
204
Q

What to check in Anemia of Chronic Dz to know if treating correctly?

A

Check inflammatory markers

205
Q

Anemia of Chronic Dz refer to nephrology or hematology if considering which therapy?

A

Parenteral recombinant EPO

206
Q

What is Heme comprised of?

A

Porpheryn ring and iron.

207
Q

Sideroblastic Anemia etiology?

A

Defect in heme synthesis. Ringed sideroblast around nucleus preventing iron from getting in.

208
Q

2 types of Sideroblastic Anemia

A

Acquired and Congenital

209
Q

Sideroblastic Anemia defect in what?

A

Defect in biosynthesis heme synthesis (common) or mitochondrial protein synthesis (less common)

210
Q

Shape of sideroblast in Sideroblastic Anemia? Causing what?

A

Ringed sideroblast. Causing ineffective erythropoiesis.

211
Q

Iron levels in Sideroblastic Anemia?

A

Iron overload!

212
Q

Sideroblastic Anemia RBCs?

A

Hypochromic, microcytic, poiklocytosis

213
Q

Sideroblastic Anemia associated with deficiency of what?

214
Q

Drugs that can cause Sideroblastic Anemia?

A

INH (TB test), chloramphenicol, Linezolid

215
Q

Sideroblastic Anemia signs and symptoms

A
  • Fatigue, dizziness, pallor

- Hepatosplenomegaly

216
Q

Sideroblastic Anemia symptoms vary depending on what?

A

Depending on the cause

217
Q

Cytic RBCs in Sideroblastic Anemia are this or this?

A

Microcytic or macrocytic

218
Q

Sideroblastic Anemia and neutropenia due to what?

A

Cu deficiency

219
Q

Sideroblastic Anemia iron studies?

A

SI=elevated
SF=elevated
TIBC=low
% Sat=elevated

220
Q

Sideroblastic Anemia and lead poisoning will show what on smear?

A

Basophillic stippling

221
Q

Diagnostic study for Sideroblastic Anemia?

A

Bone marrow biopsy. Shows ringed sideroblasts.

222
Q

What are Pappenheimber Bodies in Sideroblastic Anemia?

A

Seen with excess iron

223
Q

Where does iron ring occur in Sideroblastic Anemia?

A

In bone marrow!

224
Q

Sideroblastic Anemia tx?

A
  • Transfuse if needed

- Chelate if needed

225
Q

If Sideroblastic Anemia d/t INH?

A

B6 will fix

226
Q

Splenectomy and Sideroblastic Anemia? Contraindicated when?

A

Contraindicated if congenital

227
Q

Lead Toxicity etiology? (Hint: protoporphyrin)

A

Decreases heme synthesis. Inhibits iron binding to protoporphyrin ring and inhibits protoporphyrin synthesis.

228
Q

Lead Toxicity peripheral blood smear almost always shows what?

A

Basophilic stippling

229
Q

Lead Toxicity anemia (MCV, MCH)?

A

Hypochromic, normocytic or microcytic

Clinical Medicine 1 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions