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Pathology-Medical-Prof > Disorders of Hemoglobin > Flashcards

Disorders of Hemoglobin Flashcards

1
Q

Hb disorders name ??

A
  1. Hemoglobinopathies
  2. Thalassemia

Z-245

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2
Q

Hemoglobinopathies description?

A
  1. Qualitative abnormalities
  2. Structurally defective Hb
  3. Abnormalities in the formation of globin moiety

Z- 245

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3
Q

Thalassemia description >??

A
  1. Quantitative
  2. Reduced rate of production
  3. Dec synthesis of globin polypeptide chain

Z - 245

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3
Q

Adult Hb A2 structure ??

A

Alpha 2 Delta 2

245

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4
Q

AA no in Alpha ??

A

141

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4
Q

Hb compositionn&raquo_space;??

A

Heme + Globin protein

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5
Q

Adult Hb A Structure ??

A

Alpha 2 Beta 2

245

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6
Q

Adult Hb type ??

A
  1. Hb A
  2. Hb A2
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7
Q

Fetal Hb type ??

A
  1. Hb F
  2. Hb Bart

245

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8
Q

AA no in beta & delta ??

A

146

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9
Q

Hb bart structure ??

A

Gamma 4

245

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10
Q

Hb F Structure ??

A

Alpha 2 Gamma 2

245

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11
Q

Embryonic Hb type ??

A
  1. Hb Gower 1
  2. Hb Gower 2
  3. Hb Portland

245

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11
Q

beta delta gamma epsilon chain location ??

A

Chromosome 11

245

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11
Q

Location of Alpha chain ??

A

Chromosomes-16

245

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12
Q

S C trait ??

A

Heterogenous
1 = NORMAL
Another = Abnormal gene

246

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12
Q

Sickle cell anemia definiton ??

A

Hereditory disorders in which the red cells contain Hb-S

246- Z

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13
Q

Hb S vs Hb A ??

A

In Hb S glutamic acid is replaced by valine in 6th position of beta chain

246

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14
Q

S C disease ??

A

2 abnormal gene

246

14
Q

Type of SCA ??

A
  1. S C trait
  2. S C disease

246

15
Q

Progression of SCA ??

A
  1. Small blood vessel blockage
  2. Ischemia
  3. InFRACTION

246

16
Q

RBC fate in sickle unsickle cycle ??

A
  1. Fragile
  2. Spheorcytic

246

17
Q

S C trait occurs ??

A

Heterogenous state for the Hb S gene

246

18
Q

RBC shape in SCA ??

A

Sickled - Crescent shaped RBC

246

19
Q

MCV MCH state in SCT ??

20
Q

2 test report in SC Trait ??

A
  1. Sickle test
  2. Hb solubility test +

246

21
Q

SC Disease present with ??

A
  1. Hemolytic anemia
  2. Organ damage
  3. Episodes of pain

246

22
Q

% of Hb S in SC trait ??

23
Q

% of Hb S in SC disease ??

24
Q

Signs and symptoms of SCA ??

A
  1. Anemia
  2. Vaso occlusive crises- Occlusion of vessels by sickel cells
  3. Aplastic crises
  4. Splenic sequestration crisis
  5. Acute hypovolemia
  6. Shock
  7. Infection by Streptococcus Salmonella
  8. Growth retardation
  9. Underweight
  10. Delayed sexual maturation

247

25
Q

CBC report of SCA ??

A
  1. Hb% —– Reduced (6-9) g/dl
  2. MCV MCH MCHC = Normal

356

25
Q

Why anemia in SCA ?

Guide 357

A

S Cells are destroyed prematurely by mononuclear phagocyte system

357

26
Q

What we see in Peripheral Blood film >??

A
  1. RBC
  2. WBC
  3. Platelates
26
Q

Platelets condition in SCA ??

A

Normal // Moderately raised

356 G

27
Q

RBC features in SCA ??

A
  1. Normochromic normocytic
  2. Anisocytosis
  3. Poikilocytosis
  4. Sickle cells
  5. Target cells
  6. Howell jolly bodies

356 G

28
Q

Biochemical finidings in SCA ??

A
  1. Serum bilirubin Inc
  2. Serum & RC folate low
  3. Osmotic fragility dec
  4. Serum haptoglobin & haemopexin dec

356

28
Q

WBC features in SCA ?

A

Neutrophilic leukocytosis

356 G

28
Q

Bone marrow - Cellularity test in SCA ??

A

Hypercellularity
inc

356

29
Q

M/E ratio in SCA bone marrow test

A

Decreaased

356

30
Q

Which special cells are found in SCA RBC ??

A

Target cells

358

31
Q

Target cells composition ??

A
  1. Central =Haemoglobinized area
  2. Peripheral rim = Haemoglobinized area
  3. Between (1 & 2 ) = Non staining Clear area

357

32
Q

Conditions in which target cells are found in PBF ??

A
  1. Iron D A
  2. SCA
  3. Thalassemia
  4. Haemoglobinopathies
  5. Liver D

HITS

33
Q

Hb-S main defect ?

A

number-6 glutamic acid is replaced by valine

34
Q

Hb-C main defect ?

A

number-6 glutamic acid is replaced by lysine

34
Q

Hb-E main defect ?

A

Number-26 glutamic acid is replaced by lysine

35
Q

Hb-D-Punjab main defect ?

A

number-121 gA is replced by glutamine

36
Q

what is PCV ?

A

Volume of RBC in relation to that of whole blood

Pathology-Medical-Prof (35 decks)

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