Sickle Cell Disease

Question 1

Deoxygenated HbS →…………… → sickling of RBCs

Answer 1

polymerization

Question 2

Triggers of SCD

Answer 2

Low oxygen, dehydration, acidosis, infection

Question 3

Repeated sickling →………… → chronic hemolysis

Answer 3

membrane damage

Question 4

Which type of hemolysis accrue in SCD

Answer 4

Extravascular and intravascular hemolysis

Question 5

High erythropoietin (EPO) →………… reticulocyte count in SCD

Answer 5

increased

Question 6

All patients with SCD require………… supplementation

Answer 6

folate

Question 7

Major Clinical Manifestations

Answer 7

Major Clinical Manifestation :
- Splenic Sequestration Crisis
- Splenic Failure

Acute Chest Syndrome (ACS)
Renal Dysfunction
Musculoskeletal Complications
Stroke

Question 8

Signs of Splenic Sequestration Crisis

Answer 8

Rapid anemia, enlarged spleen, left lower quadrant pain, hypovolemic shock

Question 9

More common in children (before spleen develops fibrosis)

Answer 9

Splenic Sequestration Crisis

Question 10

Treatment of Splenic Sequestration Crisis

Answer 10

o Acute: Fluids, blood transfusion
o Definitive: Splenectomy (to prevent recurrence)

Question 11

Functional asplenia by age 4

Answer 11

Splenic Failure

Question 12

Repeated vaso-occlusion → splenic fibrosis

Answer 12

Splenic failure

Question 13

↑ Risk of infections with encapsulated organisms

Answer 13

Splenic Failure

Question 14

Prophylaxis form encapsulated bacteria

Answer 14

o Penicillin until age 5 (if allergic → erythromycin)
o Vaccinations: Pneumococcal (PCV13, PPSV23), meningococcal, Hib,
influenza

Question 15

Leading cause of death in adults with SCD

Answer 15

Acute Chest Syndrome (ACS)

Question 16

Triggers for Acute Chest Syndrome (ACS)

Answer 16

Infection (pneumonia), dehydration, hypoxia

Question 17

Symptoms for Acute Chest Syndrome (ACS

Answer 17

Fever, chest pain, wheezing, cough, hypoxemia, respiratory distress

Question 18

Treatment of ACS

Answer 18

o Fluids + pain management (like pain crisis)
o Oxygen, antibiotics, bronchodilators
o Transfusion if severe

Question 19

Vaso-occlusion of pulmonary microvasculature

Answer 19

Acute Chest Syndrome (ACS)

Question 20

Vaso-occlusion in renal medulla → hypoxia + hyperosmolarity (promotes sickling)

Answer 20

Renal Dysfunction

Question 21

Complications of Vaso-occlusion in renal medulla

Answer 21

o Hyposthenuria: Inability to concentrate urine (polyuria, nocturia)
o Hematuria
o Increased risk of CKD

Question 22

Sickle cell trait (AS): ↑ Risk of …………….

Answer 22

renal medullary carcinoma

Question 23

Vast active occlusion can cause Musculoskeletal Complications include

Answer 23

Avascular necrosis (AVN)
o Bone collapse (most commonly femoral or humeral head)

Osteomyelitis

Question 24

Most common cause of Osteomyelitis in SCD

Answer 24

Salmonella

Question 25

MCC of Osteomyelitis In non-SCD:

Answer 25

Staphylococcus aureus

Question 26

Common in pediatric patients with SCD

Answer 26

Stroke

Question 27

Treated with exchange transfusion (removes sickled RBCs, replaces with normal RBCs

Answer 27

Stroke

Question 28

Hemoglobin electrophoresis, HPLC, or isoelectric focusing in SCD

Answer 28

o Sickle cell disease (SS): HbS >85%, no HbA o Sickle cell trait (AS): HbS 30-40%, HbA present

Question 29

Chronic Management of SCD

Answer 29

Hydroxyurea Bone Marrow Transplant (BMT) o Only curative treatment

Question 30

Myelosuppression (bone marrow suppression side effect of ??

Question 31

General Management of SCD

Answer 31

 Immunizations (pneumococcal, meningococcal, Hib, influenza)  Folate supplementation  Antibiotic prophylaxis (oral penicillin until age 5)

Question 32

Mild hemolysis, splenomegaly

Answer 32

Mild hemolysis, splenomegaly Glutamate → Lysine mutation