1. Hemoglobinopathies 2. Thalassemia Z-245

1. Hb Gower 1 2. Hb Gower 2 3. Hb Portland 245

Disorders of Hemoglobin Flashcards by Istiak Mahmud Siam

Hb disorders name ??

Hemoglobinopathies
Thalassemia

Z-245

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Hemoglobinopathies description?

Qualitative abnormalities
Structurally defective Hb
Abnormalities in the formation of globin moiety

Z- 245

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Thalassemia description >??

Quantitative
Reduced rate of production
Dec synthesis of globin polypeptide chain

Z - 245

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Adult Hb A2 structure ??

Alpha 2 Delta 2

245

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AA no in Alpha ??

141

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Hb compositionn&raquo_space;??

Heme + Globin protein

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Adult Hb A Structure ??

Alpha 2 Beta 2

245

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Adult Hb type ??

Hb A
Hb A2

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Fetal Hb type ??

Hb F
Hb Bart

245

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AA no in beta & delta ??

146

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Hb bart structure ??

Gamma 4

245

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Hb F Structure ??

Alpha 2 Gamma 2

245

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Embryonic Hb type ??

Hb Gower 1
Hb Gower 2
Hb Portland

245

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beta delta gamma epsilon chain location ??

Chromosome 11

245

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Location of Alpha chain ??

Chromosomes-16

245

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S C trait ??

Heterogenous
1 = NORMAL
Another = Abnormal gene

246

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Sickle cell anemia definiton ??

Hereditory disorders in which the red cells contain Hb-S

246- Z

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Hb S vs Hb A ??

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

246

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S C disease ??

2 abnormal gene

246

Type of SCA ??

S C trait
S C disease

246

Progression of SCA ??

Small blood vessel blockage
Ischemia
InFRACTION

246

RBC fate in sickle unsickle cycle ??

Fragile
Spheorcytic

246

S C trait occurs ??

Heterogenous state for the Hb S gene

246

RBC shape in SCA ??

Sickled - Crescent shaped RBC

246

MCV MCH state in SCT ??

Normal

2 test report in SC Trait ??

1. Sickle test 2. Hb solubility test + | 246

SC Disease present with ??

1. Hemolytic anemia 2. Organ damage 3. Episodes of pain | 246

% of Hb S in SC trait ??

40%

% of Hb S in SC disease ??

90% | 246

Signs and symptoms of SCA ??

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

CBC report of SCA ??

1. Hb% ----- Reduced (6-9) g/dl 2. MCV MCH MCHC = Normal | 356

Why anemia in SCA ? | Guide 357

S Cells are destroyed prematurely by mononuclear phagocyte system | 357

What we see in Peripheral Blood film >??

1. RBC 2. WBC 3. Platelates

Platelets condition in SCA ??

Normal // Moderately raised | 356 G

RBC features in SCA ??

1. Normochromic normocytic 2. Anisocytosis 3. Poikilocytosis 4. Sickle cells 5. Target cells 6. Howell jolly bodies | 356 G

Biochemical finidings in SCA ??

1. Serum bilirubin Inc 2. Serum & RC folate low 3. Osmotic fragility dec 4. Serum haptoglobin & haemopexin dec | 356

WBC features in SCA ?

Neutrophilic leukocytosis | 356 G

Bone marrow - Cellularity test in SCA ??

Hypercellularity inc | 356

M/E ratio in SCA bone marrow test

Decreaased | 356

Which special cells are found in SCA RBC ??

Target cells | 358

Target cells composition ??

1. Central =Haemoglobinized area 2. Peripheral rim = Haemoglobinized area 3. Between (1 & 2 ) = Non staining Clear area | 357

Conditions in which target cells are found in PBF ??

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

Hb-S main defect ?

number-6 glutamic acid is replaced by valine

Hb-C main defect ?

number-6 glutamic acid is replaced by lysine

Hb-E main defect ?

Number-26 glutamic acid is replaced by lysine

Hb-D-Punjab main defect ?

number-121 gA is replced by glutamine

what is PCV ?

Volume of RBC in relation to that of whole blood