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Hematology 2 Lec > THALASSEMIA > Flashcards

THALASSEMIA Flashcards

1
Q

▪ results of impaired (deficient) globin chain synthesis

A

THALASSEMIA

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2
Q

▪ may affect either the alpha or beta chain synthesis

A

THALASSEMIA

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3
Q

THALASSEMIA ▪ diverse group of genetic disorders due to:

A

a. quantitative reduction in globin chain synthesis for hemoglobin

b. formation of structurally abnormal hemoglobins formed from normal globin chains or parts of normal chains

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4
Q

a. quantitative reduction in globin chain synthesis for hemoglobin

A

• alpha (a)
• beta (ß)
• gamma (y)
• delta (δ)

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5
Q

b. formation of structurally abnormal hemoglobins formed from normal globin chains or parts of normal chains

A

• Hb H (ß4)
• Hb Barts (y4)
• Hb Lepore
• Hb Constant Spring (presents with a thalassemic clinical picture)

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6
Q

▪ decreased or non-existent production of one or more globin chain type

A

THALASSEMIA

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7
Q

• most common thalassemias:

A
  • α-thalassemia: ↓ α-chain(s)
  • ß-thalassemia: ↓ ß-chain(s)
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8
Q

▪ human hemoglobin may contain any of seven different globin polypeptide chains

A
  1. alpha (α)
  2. beta (ß)
  3. G gamma (G γ)
  4. A gamma (A γ)
  5. delta (δ)
  6. epsilon (ɛ)
  7. zeta (ζ)
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9
Q

▪ globin rearrangement usually occurs in:

A

• chromosome 11

• chromosome 16

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10
Q

• chromosome 11:

A
  • beta (ß)
  • gamma (γ)
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11
Q

• chromosome 16:

A
  • alpha (α)
  • zeta (ζ)
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12
Q
  • gamma (γ): two types of y chains produced (differ in the 136th position)
A
  1. G gamma (Gγ)
  2. A gamma (Aγ)
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13
Q
  • amino acid: glycine
A
  1. G gamma (Gγ)
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14
Q
  • amino acid: alanine
A
  1. A gamma (Aγ)
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15
Q
  • delta (δ) - epsilon (ɛ)
A
  1. A gamma (Aγ)
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16
Q

141

A

alpha

zeta

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17
Q

unknown

A

theta

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18
Q

146

A

beta

delta

gamma

epsilon

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19
Q

4; Ch 16

A

alpha

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20
Q

2; Ch 11

A

beta

delta

gamma

epsilon

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21
Q

2; Ch 16

A

zeta

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22
Q

double

A

alpha

gamma

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23
Q

single

A

beta

delta

epsilon

zeta

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24
Q

: embryonic chains

A

*Epsilon and zeta

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25
Q

: embryonic counterpart of alpha

A

*Zeta

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26
Q

: embryonic counterpart of beta, delta, and gamma

A

*Epsilon

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27
Q

▪ 1 globin

A

GLOBIN STRUCTURE

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28
Q

▪ protein portion composed of 2 sets (dimer) of 2 different polypeptide chains

A

GLOBIN STRUCTURE

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29
Q

▪ determines the type of hemoglobin

A

GLOBIN STRUCTURE

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30
Q

2 ζ 2 ɛ

A

Gower I

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31
Q

embryonic Hbs

A

Gower I
Gower II
Portland 2

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32
Q

2 α 2 ɛ

A

Gower II

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33
Q

ζ 2 γ

A

Portland 2

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34
Q

2 α 2 γ

A

Fetal (Hb F)

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35
Q

2 α 2 ß

A

major Hb A1

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36
Q

A2 2 α 2 δ

A

minor Hb

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37
Q

newborns and adults

A

Fetal (Hb F)

major Hb A1

minor Hb A2

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38
Q

Hemoglobin concentrations in adults

A

major Hb A1: 92-95%

Hb A2: 2-3%

Hb F: 1-2%

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39
Q

• most potential Hb incorporated on RBCs to transport oxygen to the tissues

A

major Hb A1: 92-95%

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40
Q

• compensation in cases of abnormalities affecting Hb A1

A

Hb A2: 2-3%

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41
Q

▪ [?] anemia

A

microcytic and hypochromic

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42
Q

▪ RBC count:

A

elevated (↑)

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43
Q

▪ abnormalities:

A

• nucleated RBCs
• microcytosis
• hypochromia
• anisocytosis
• poikilocytosis
• polychromasia
• basophilic stippling
• target cells (predominant)
• leukocytes and platelets

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44
Q
  • variations in the shades of cell colors
A

• polychromasia

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45
Q
  • caused by a combination of the affinity of hemoglobin ribosomal RNA
A

• polychromasia

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46
Q

(predominant)

A

• target cells

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47
Q

: not affected- display of diffused bluish-gray tint of RBCs

A

• leukocytes and platelets

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48
Q

▪ concurrent iron deficiency in thalassemia causes:

A

• lower concentrations of Hb A2 and Hb H

• lower serum iron and iron stores

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49
Q
  • may mask diagnosis of heterozygous ß thalassemia and Hb H disease, respectively
A

• lower concentrations of Hb A2 and Hb H

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50
Q
  • Hb H inclusions may be more difficult to detect in red cells
A

• lower concentrations of Hb A2 and Hb H

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51
Q

▪ diagnosis: in cases of iron deficient individuals, [?] should first be repleted before performing diagnostic tests for thalassemia

A

iron deficient iron stores

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52
Q

▪ on the short arm of chromosome 16

A
  1. ALPHA GENES
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53
Q

: 4 genes (2 genes each inherited from parents)

A

▪ (αα/αα)

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54
Q

• 2 copies of α-globin gene per chromatid =

A

4 genes per diploid cell (αα/αα)

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55
Q

▪ nomenclature:

A

• 1 gene deletion: -α

• 2 gene deletion: (–)

• 3 gene deletion: –/-α or α-/–

• 4 gene deletion: –/– • non-functional α-genes: present but nonfunctional hence do not code for globin synthesis (partially suppressed gene)

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56
Q

• 1 gene deletion:

A

-α

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57
Q

• 2 gene deletion:

A

(–)

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58
Q

• 3 gene deletion:

A

–/-α or α-/–

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59
Q

• 4 gene deletion:

A

–/–

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60
Q

: present but nonfunctional hence do not code for globin synthesis (partially suppressed gene)

A

• non-functional α-genes

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61
Q

• non-functional α-genes:

A
    • superscript (+ )
  • example: αα+
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62
Q

: -α - α-thalassemia 2 or α+ thalassemia

A

• 1 gene deletion

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63
Q
  • αα/-α or α-/αα
A

• 1 gene deletion

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64
Q

: (–) - α-thalassemia 1 or αo thalassemia

A

• 2 gene deletion

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65
Q
  • homozygous:
A

αα/– or –/αα

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66
Q
  • heterozygous (1 α-gene deleted per parent):
A

α-/α- or -α/-α

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67
Q

▪ deficiency in the synthesis of alpha-globin chains

A

ALPHA THALASSEMIA

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68
Q

▪ each individual has two sets of two alpha genes

A

ALPHA THALASSEMIA

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69
Q

▪ usual mechanism of suppression: gene deletion

A

ALPHA THALASSEMIA

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70
Q

• causes suppression of all four genes leading to complete suppression of alpha chain synthesis

A

gene deletion

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71
Q

• 2 haplotypes:

A
  1. one gene deletion: α-thalassemia 2 or α+ thalassemia
  2. double gene deletion: αthalassemia 1 or αo thalassemia (more severe)
72
Q

: α-thalassemia 2 or α+ thalassemia

A
  1. one gene deletion
73
Q

: αthalassemia 1 or αo thalassemia (more severe)

A
  1. double gene deletion
74
Q

Alpha Thalassemia Variants

A
  1. Silent carrier/α-Thalassemia minor
  2. α-Thalassemia trait/α-Thalassemia minor
  3. Hb H Disease
  4. Bart’s Hydrops fetalis (Hb Barts)
75
Q

▪ 1/4 α-gene deleted

A
  1. Silent carrier/α-Thalassemia minor
76
Q

▪ heterozygous: α-/αα (α+ )

A
  1. Silent carrier/α-Thalassemia minor
77
Q

▪ remaining 3 α-genes are still able to regulate the synthesis of normal amounts of α-chains

A
  1. Silent carrier/α-Thalassemia minor
78
Q

▪ no signs and symptoms observed

A
  1. Silent carrier/α-Thalassemia minor
79
Q

▪ 2/4 α-genes deleted

A
  1. α-Thalassemia trait/α-Thalassemia minor
80
Q
  1. α-Thalassemia trait/α-Thalassemia minor
    ▪ genotypes:
A

• homozygous: αα/– or –/αα
• heterozygous: α-/α- (1 gene deleted per parent)

81
Q

▪ can still synthesize α-chains but insufficient

A
  1. α-Thalassemia trait/α-Thalassemia minor
82
Q

▪ MILD signs and symptoms

A
  1. α-Thalassemia trait/α-Thalassemia minor
83
Q

▪ 3/4 α-genes deleted

A
  1. Hb H Disease
84
Q

• α 0 / α+ (–/-a)

A
  1. Hb H Disease
85
Q

▪ remaining 1 gene is unable to dictate the synthesis of the required concentration of α-chain

A
  1. Hb H Disease
86
Q

▪ ↓ α-chain = ↑ ß-chain → tetramer ß4 of hemoglobin

A
  1. Hb H Disease
87
Q

• 1 Hb consists of 4 ß-chains

A

▪ Hb H

88
Q

• unstable

A

▪ Hb H

89
Q

• leads to formation of Heinz bodies which makes RBCs become rigid and destroyed by spleen

A

▪ Hb H

90
Q

: supravitally stained with brilliant cresyl blue (BCB)

A

Heinz bodies

91
Q

• phenotype similar to Hb H disease

A

▪ Hb H-Constant Spring Disease (Hb H/CS)

92
Q
  • inheritance of Hb CS causes a deficit in normal α chains, and when it is inherited along with a double a-gene deletion, it produces a disorder similar to Hb H disease
A

▪ Hb H-Constant Spring Disease (Hb H/CS)

93
Q

• caused by compound heterozygous inheritance of Hb Constant Spring (Hb CS) and α 0 thalassemia (–/α CSα)

A

▪ Hb H-Constant Spring Disease (Hb H/CS)

94
Q

• more severe hemolysis than in the typical threeα gene deletion Hb H disease

A

▪ Hb H-Constant Spring Disease (Hb H/CS)

95
Q

▪ 4/4 α-genes deleted (–/–) (α0 )

A
  1. Bart’s Hydrops fetalis (Hb Barts)
96
Q

▪ no synthesis of α-chains resulting in the production of Hb Barts (tetramer γ4)

A
  1. Bart’s Hydrops fetalis (Hb Barts)
97
Q

: x α-chains

A

• Hb F

98
Q

↑ γ-chains

A

• Hb F

99
Q

: has a high affinity for oxygen and thus will not effectively release it to the tissues (no oxygen delivered)

A

• Hb Barts

100
Q
  • suffers still-birth
A

• fetus

101
Q
  • does not survive (dies inside the uterus)
A

• fetus:

102
Q

: swollen (enlarged) head

A
  • hydrops fetalis
103
Q

▪ can be manifested in as early as the fetal life

A
  1. Bart’s Hydrops fetalis (Hb Barts)
104
Q

• no synthesis of Hb A and Hb F

A
  1. Bart’s Hydrops fetalis (Hb Barts)
105
Q

Classifications of Alpha Thalassemia

A

One-gene deletion (αα/α)

Two gene deletion (-α/α; αα/–)

Three-gene deletion (-α/-)

106
Q

Silent Carrier/ α-Thal minor

A

One-gene deletion (αα/α)

107
Q

▪ hematology: normal with few target cells and elliptocyte

A

One-gene deletion (αα/α)

108
Q

▪ asymptomatic

A

One-gene deletion (αα/α)
Two gene deletion (-α/α; αα/–)

109
Q

AlphaThalasse mia Trait/ αThal minor

A

Two gene deletion (-α/α; αα/–)

110
Q

▪ mild anemia

A

Two gene deletion (-α/α; αα/–)

111
Q

▪ microcytic hypochromic

A

Two gene deletion (-α/α; αα/–)

Three-gene deletion (-α/-)

112
Q

▪ elliptocytes

A

Two gene deletion (-α/α; αα/–)

113
Q

▪ target cells

A

Two gene deletion (-α/α; αα/–)

114
Q

▪ Hb A (60%)

A

Two gene deletion (-α/α; αα/–)

115
Q

▪ Hb Barts (5- 10%)

A

Two gene deletion (-α/α; αα/–)

116
Q

Hb H Disease

A

Three-gene deletion (-α/-)

117
Q

▪ Hb H inclusions (Heinz bodies)

A

Three-gene deletion (-α/-)

118
Q

▪ high retics

A

Three-gene deletion (-α/-)

119
Q

▪ 30% Hb H (ß4) – precipitated into Heinz bodies

A

Three-gene deletion (-α/-)

120
Q

▪ Constant Spring disease (Hb H/CS)

A

Three-gene deletion (-α/-)

121
Q

– precipitated into Heinz bodies

A

▪ 30% Hb H (ß4)

122
Q

• 6% Hb Barts

A

α-Thal minor (one & two gene deletion)

123
Q
  • asymptomatic (accidentally discovered) in adults (not diagnostic)
A

α-Thal minor (one & two gene deletion)

124
Q
  • considered diagnostic in newborns
A

α-Thal minor (one & two gene deletion)

125
Q

▪ short arm of chromosome 11

A
  1. BETA GENES
126
Q

▪ one copy per chromatid

A
  1. BETA GENES
127
Q

• total: 2 ß-genes per diploid cell

A
  1. BETA GENES
128
Q

• ß/ß

A
  1. BETA GENES
129
Q

: gene cannot fully synthesize required number of ß-chains (decreased production but not fully deficit)

A

▪ + superscript (+ )

130
Q

: deleted gene (no production)

A

▪ 0 superscript (0 )

131
Q

: reduced ß-chain production

A

▪ ß +

132
Q

: no ß-chain production

A

▪ ß 0

133
Q

: δ and ß-gene deletion

A

▪ (δß)0

134
Q

: Hb Lepore designation

A

▪ (δß)Lepore

135
Q

• nonhomologous meiotic crossing over between δ and ß globin genes (exchanging of genetic material or traits) on chromosome 11

A

▪ (δß)Lepore

136
Q

▪ unbalanced globin chain synthesis due to a lack of, or to the reduced production of, ß chains

A

BETA THALASSEMIA

137
Q

• causes an excess of α chains (very unstable)

A

BETA THALASSEMIA

138
Q
  • normally, production of α and ß chains should be proportionate (equal production for them to be paired – Hb A1)
A

BETA THALASSEMIA

139
Q

BETA THALASSEMIA
• minor imbalance:

A

unpaired α chains are simply removed by proteolysis during erythroid maturation

140
Q

BETA THALASSEMIA
• major imbalance:

A

overwhelmed/massive number of unpaired α chains (excess free α chains) precipitate (results to Heinz bodies), causing severe erythrocyte dysfunction (rigid RBCs)

141
Q
  • defective red cells are destroyed by bone marrow macrophages causing ineffective erythropoiesis and a massively enlarged erythron
A

BETA THALASSEMIA

142
Q

▪ characterized by a deficiency in Beta-globin chain synthesis

A

BETA THALASSEMIA

143
Q

▪ each individual has one set of two beta genes

A

BETA THALASSEMIA

144
Q

▪ suppression or absence of these beta genes results to deficient beta chain synthesis

A

BETA THALASSEMIA

145
Q

Classifications of Beta Thalassemia

A

ß-Thal Minor/Trait (Cooley’s Trait)

ß-Thal Intermedia

ß-Thal Major (Cooley’s Anemia)

146
Q

ß 0 /ß0

A

ß-Thal Major (Cooley’s Anemia)

147
Q

severe

A

ß-Thal Major (Cooley’s Anemia)

148
Q

ß/ß+ or ß/ß0

A

ß-Thal Minor/Trait (Cooley’s Trait)

149
Q

silent

A

ß-Thal Minor/Trait (Cooley’s Trait)

150
Q

ß + /ß+ or ß+ /ß0

A

ß-Thal Intermedia

151
Q

moderate

A

ß -Thal Intermedia

152
Q

▪ 1/2 normal ß-gene: still able to synthesize at least enough ß-chains

A

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)

153
Q

• other ß-gene: either deleted or suppressed

A

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)

154
Q

▪ still able to produce sufficient quantities of HbA1

A

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)

155
Q

▪ hemoglobin levels: slightly low

A

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)

156
Q

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)
▪ blood smear:

A

• shows similar morphology as in Thalassemia Major or Intermedia
• mild microcytic-hypochromic anemia
• basophilic stippling
• target cells

157
Q

ß-Thalassemia Minor/Cooley’s Trait (ßO /ß)
▪ Hb F level:
▪ Hb A2 level:
▪ Hb A1 level:

A

▪ Hb F level: 2-3%
▪ Hb A2 level: elevated
▪ Hb A1 level: decreased

158
Q

▪ caused by a partial suppression of the Beta genes

A

ß-Thalassemia Intermedia (ß+ /ß+ )

159
Q

▪ lesser production of Hb A1

A

ß-Thalassemia Intermedia (ß+ /ß+ )

160
Q

▪ symptoms are similar with that of Beta Thalassemia major, but depend on the extent of gene suppression

A

ß-Thalassemia Intermedia (ß+ /ß+ )

161
Q

▪ milder than Beta Thalassemia major

A

ß-Thalassemia Intermedia (ß+ /ß+ )

162
Q

ß-Thalassemia Intermedia (ß+ /ß+ )
▪ genotypes:

A

• homozygous: ß + /ß+
• heterozygous: ß + /ß0

163
Q

▪ complete lack of Beta globin production

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

164
Q

• markedly decreased or absence of ßchain synthesis (resulting in excess α chains)→ no production of Hb A1

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

165
Q

-[?]Hb F becomes increased up to [?] to compensate for the absence of Hb A1

A

1-3%

40-60%

166
Q
  • Hb F can go as high as [?]
A

90-98%

167
Q

▪ most affected patients exhibit retarded growth with mongoloid facial features and severe anemia

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

168
Q

▪ marked by characteristic changes in RBC morphology:

A

• microcytosis
• hypochromia
• anisocytosis
• poikilocytosis
• increased nucleated RBCs
• basophilic stippling
• Pappenheimer bodies
• Heinz bodies (resulted from precipitated unpaired α chains)
• numerous target cells, Howell-Jolly bodies, and siderocytes
• ↑serum iron and bilirubin

169
Q

▪ HbF level: 40-60% (elevated)

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

170
Q

▪ requires repeated/continuous blood transfusion

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

171
Q

▪ Hb Lepore:

A

(δß)Lepore/(δß)Lepore

172
Q

• composed of two normal α chains and two abnormal nonα chains formed by fusion of the N-terminal end of a δ chain and the C-terminal end of a ß chain

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

173
Q

• caused by nonhomologous meiotic crossing over between the δ and ß globin gene loci

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

174
Q

• remaining portions of the δ and ß genes form an antiLepore gene (to stop all portions in becoming abnormal)

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

175
Q

• chromosome bearing the Lepore δß fusion gene lacks intact δ and ß genes

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

176
Q

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )
• 3 different Lepore hemoglobins: each differs in the point at which crossing over occurs

A
  1. Hb Lepore-Baltimore
  2. Hb Lepore-Boston (most common)
  3. Hb Lepore-Hollandia
177
Q

• all Hbs Lepore are ineffectively synthesized, causing a ß thalassemia syndrome

A

ß-Thalassemia Major/Cooley’s Anemia (ßO /ßO )

Hematology 2 Lec (29 decks)

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