L1: Anemia - Hemolytic Anemia (3)

Question 1

Def of Sickle Cell Anemia

Answer 1

Congenital chronic hemolytic anemia due to the presence of an abnormal Hb

Question 2

Incidence of Sickle Cell Anemia

Answer 2

• It is common in black races.
• It appears after the age of 6 months

Question 3

Pathogenesis of Sickle Cell Anemia

Answer 3

Question 4

Pathogenesis of Sickle Cell Anemia

• Inheritance

Answer 4

Intermediate inheritance

(incomplete dominance)

Question 5

Pathogenesis of Sickle Cell Anemia

• Process

Answer 5

Question 6

CP of Sickle Cell Anemia

Answer 6

Question 7

CP of Sickle Cell Anemia

• Chronic Hemolyic Anemia

Answer 7

…..

Question 8

CP of Sickle Cell Anemia

• Crises

Answer 8

Question 9

Crises in CP of Sickle Cell Anemia

• Thrombotic (Vaso-Occlusive)

Answer 9

Question 10

Crises in CP of Sickle Cell Anemia

• Hemolytic (Sequestration)

Answer 10

Sudden progressive destruction of RBCs in the liver & spleen leading to:
- Shock
- Massive HSM

Question 11

Crises in CP of Sickle Cell Anemia

• Hyper-Hemolytic

Answer 11

• It’s characterized by excessive hemolysis (C/P of acute hemolytic anemia)
• The patient has also G6PD deficiency

Question 12

Crises in CP of Sickle Cell Anemia

• Megaloblastic

Answer 12

• It is usually due to vitamin B12 deficiency

Question 13

Crises in CP of Sickle Cell Anemia

• Aplsatic

Answer 13

• Sudden attack of B.M. aplasia which recover after 2 weeks.
• It is usually due to Parvovirus B19

Question 14

CP of Sickle Cell Anemia

• Spleen

Answer 14

• Splenic infarction in sickle cell anemia → Auto-splenectomy
• Good hydration is needed to prevent thrombosis

Question 15

Complications of Sickle Cell Anemia

Answer 15

1. Short stature & delayed puberty
2. Heart & Renal failure.
3. Gall stones
4. Leg ulcers

Question 16

INVx in Sickle Cell Anemia

Answer 16

Question 17

INVx in Sickle Cell Anemia

• PBE

Answer 17

Question 18

INVx in Sickle Cell Anemia

• BMA

Answer 18

• Erythroid hyperplasia.
• inc. iron stores (Prussian blue stain)

Question 19

INVx in Sickle Cell Anemia

• Sickling Test

Answer 19

Examine RBCs under low O2 tension —> Sickling

Question 20

INVx in Sickle Cell Anemia

• Hb Electrophoresis

Answer 20

Most Specific

• HbS, HbA2 & HbF (but no HbA)

Question 21

TTT of Sickle Cell Anemia

• Between Attacks

Answer 21

Question 22

TTT of Sickle Cell Anemia Between Attacks

• Blood Transfusion

Answer 22

if Hb decreases < 6 gm/dl

Question 23

TTT of Sickle Cell Anemia Between Attacks

• Hydroxyurea (Hb F inducer)

Answer 23

In cases of recurrent attacks of thrombotic crisis.

Question 24

TTT of Sickle Cell Anemia Between Attacks

• Spleenectomy

Answer 24

(indicated in the following conditions):
- Pressure symptoms.
- Hypersplenism.
- Repeated sequestration crisis.

Question 25

TTT of **Sickle Cell Anemia** - During Attack

Answer 25

Question 26

Prognosis of **Sickle Cell Anemia**

Answer 26

- The condition improved with age. - The prognosis is guarded by cerebral artery occlusion

Question 27

Pathogensis of **G6PD Deficiency**

Answer 27

Question 28

Incidence of **G6PD Deficiency**

Answer 28

Question 29

Classififcation of **G6PD Deficiency**

Answer 29

Question 30

PPT Factors for **G6PD Deficiency**

Answer 30

Question 31

PPT Factors for **G6PD Deficiency** - Favism

Answer 31

Question 32

PPT Factors for **G6PD Deficiency** - Drugs

Answer 32

Question 33

PPT Factors for **G6PD Deficiency** - Severe Infection

Answer 33

Phagocytes produce H202 and 02 radicals

Question 34

CP of **G6PD Deficiency**

Answer 34

Question 35

CP of **G6PD Deficiency** - Between Attacks

Answer 35

Normal

Question 36

CP of **G6PD Deficiency** - During Attacks

Answer 36

Question 37

CP of **G6PD Deficiency** - Recurrent Severe Infections

Answer 37

......

Question 38

CP of **G6PD Deficiency** - Duration of Hemolysis

Answer 38

Question 39

INVx for **G6PD Deficiency**

Answer 39

Question 40

INVx for **G6PD Deficiency** - PBE

Answer 40

Question 41

INVx for **G6PD Deficiency** - Urine Analysis

Answer 41

**During Attack** - Hemoglobinuria

Question 42

INVx for **G6PD Deficiency** - Enzyme Assay

Answer 42

Question 43

INVx for **G6PD Deficiency** - CBC

Answer 43

**after Attack** - Completely normal (except in variants with chronic hemolysis)

Question 44

DDx of **G6PD Deficiency**

Answer 44

Other causes of dark colored urine (e.g. hepatitis) - Favism is characterized by the presence of pallor

Question 45

TTT of **G6PD Deficiency** - During Attack (Curative)

Answer 45

Question 45

TTT of **G6PD Deficiency**

Answer 45

Question 46

TTT of **G6PD Deficiency** - Between Attacks (Prophylaxis)

Answer 46

- Avoid the precipitating agents in susceptible patients. - Vitamin E: protects against hemolysis

Question 47

Def of **Hereditary Spherocytosis**

Answer 47

Congenital chronic hemolytic anemia Dz defect in cell membrane of RBCS.

Question 48

Etiology of **Hereditary Spherocytosis**

Answer 48

Question 49

Inheritance in **Hereditary Spherocytosis**

Answer 49

Question 50

CP of **Hereditary Spherocytosis**

Answer 50

Question 51

CP of **Hereditary Spherocytosis** - Asymptomatic

Answer 51

No Symptoms

Question 52

CP of **Hereditary Spherocytosis** - Symptomatic

Answer 52

Question 53

INVx for **Hereditary Spherocytosis**

Answer 53

Question 54

INVx for **Hereditary Spherocytosis** - PBE

Answer 54

Question 55

INVx for **Hereditary Spherocytosis** - BMA

Answer 55

Question 56

INVx for **Hereditary Spherocytosis** - Osmotic Fragility Test

Answer 56

**Most Specific** Measures the ability of RBCs to resist hemolysis * When exposed to different degrees of salt dilution

Question 57

INVx for **Hereditary Spherocytosis** - Flowcytometry

Answer 57

With eosin-5-maleimide

Question 58

INVx for **Hereditary Spherocytosis** - Acidified Glycerol Lysis Test

Answer 58

* RBCs are incubated in a hypotonic solution with glycerol. * Spherocytes lyse more quickly than normal RBCs → +ve result

Question 59

INVx for **Hereditary Spherocytosis** - Abdominal US

Answer 59

* For detection of gallbladder stones

Question 60

TTT of **Hereditary Spherocytosis**

Answer 60

Question 61

TTT of **Hereditary Spherocytosis** - Spleenectomy

Answer 61

Question 62

Spleenectomy in TTT of **Hereditary Spherocytosis** - Indications - Precautions

Answer 62

Question 63

TTT of **Hereditary Spherocytosis** - Exchange Transfusion

Answer 63

For neonatal jaundice

Question 64

Spleenectomy in TTT of **Hereditary Spherocytosis** - Blood Transfusion

Answer 64

If Hb < 6 gm/di

Question 65

Spleenectomy in TTT of **Hereditary Spherocytosis** - Folic Acid

Answer 65

....

Question 66

Remaining?????

Answer 66

....