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Hematology I > Thalassemia > Flashcards

Thalassemia Flashcards

1
Q

characteristic of thalassemia

A

untreated beta

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2
Q

described 4 children with thalassemia

A

cooley & lee

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3
Q

other name of thalassemia major

A

cooley’s anemia

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4
Q
  • autopsy
  • high incidence of mediterranean descent
  • greek: thalassic (great sea)
A

Whipple & Bradford

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5
Q
  • characterized by a reduction or total absence of synthesis of one or more of the globin chains of hemoglobin
A

thalassemia

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6
Q

total lack of alpha- or beta- globin production

A

thalassemia major

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7
Q

production at a decreased rate is known as

A

thalassemia minor

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8
Q

pathophysiology of thalassemia

A
  • decrease Hgb synthesis (microcytic, hypochromic)

- imbalance of globin chains (damage to RBC)

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9
Q

in thalassemia syndromes, erythrocyte indices generally indicate a

A

microcytic, hypochromic anemia

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10
Q

erythrocyte abnormalities in thalassemia include

A
  • nucleated red cells
  • microcytosis
  • hypochromia
  • anisocytosis
  • poikilocytosis
  • polychromasia
  • basophilic stippling
  • target cells
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11
Q

associated w. a decrease in the production of alpha chains

A

Alpha thalassemia

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12
Q

associated w. a decrease in the production in beta cha

A

beta-thalassemia

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13
Q
  • gamma chains are more stable, do not precipitate but form Hb bart
  • beta-gamma switching
  • RBC age in circulation forming inclusion
A

Mechanisms of alpha-thalassemia

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14
Q

-very high affinity to oxygen, cannot release oxygen

A

Hb bart

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15
Q

excess bata chain in a-thalassemia

A

Hb H

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16
Q

single gene deletion of thalassemia

A

silent carrier state (normal lab test)

17
Q

two gene deletion of thalassemia

A

alpha-thalassemia trait or minor

18
Q

three gene deletion of thalassemia

A

Hb H disease

19
Q

four gene deletion of thalassemia

A

Hemoglobin Bart’s (hydrops fetalis)

20
Q
  • composed of 4 beta chains

- resemble golf ball because of the pitted inclusion

A

HbH

21
Q

pitted golf ball appearance of Hb is made possible by staining with

A

brilliant cresyl blue

22
Q

generally occur in multiples & cover the cell surface

A

Hb inclusion

23
Q

are usually eccentrically located & there are very few cell in Hb H

A

Heinz bodies

24
Q
  • lethal disorder (infant are usually stillborn or die w/ in hours of birth
  • Hb Barts
A

Barts hydrops fetalis

25
Q

lab results of barts hydrops fetalis in PBS

A

hypochromia, variable reticulocytosis, nRBCs

26
Q

lab results of barts hydrops fetalis in Hb electrophoresis

A

fast moving ban (alkaline pH)

27
Q
  • decrease beta chain–> accumulation of alpha chain
  • alpha chain precipitate
  • RBC phagocytized in the BM
A

mechanism of b-thalassemia (ineffective erythropoiesis)

28
Q
  • symptoms appear 6 months of age

- beta gamma switching

A

mechanism of b-thalassemia (extravascular hemolysis)

29
Q

severe anemia accompanied by iron overload

A

Thalassemia major (Cooley’s anemia)

30
Q

moderate anemia

A

Thalassemia intermedia

31
Q

asymptomatic may or may not produce mild anemia

A

Thalassemia minor (Cooley’s trait)

32
Q

causes no detectable clinical or routine laboratory abnormalities

A

Thalassemia minima

33
Q

physical abnormalities of beta thalassemia major

A
  • radiograph of long bone (lacy or lucent appearance)
  • radiograph of skull ( hair on end appearance)
  • frontal bossing, cheekbones, upper jaw
  • iron accumulation
34
Q

prominence of forehead

A

frontal bossing

35
Q

beta thalassemia major in children

A

growth retardation

- absence of sexual maturity

36
Q

beta thalassemia major in adults

A
  • cardiomyopathy
  • fibrosis
  • cirrhosis
  • dysfunction of exocrine glands
37
Q
  • transfusion dependent

- cardiac failure may cause in teens

A

beta thalassemia major

38
Q

therapy for beta thalassemia major

A

iron chelation therapy

39
Q

in beta thalassemia major there is an increased

A

Hb A2 & Hb F

Hematology I (11 decks)

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