Sickle Cell Disease
Also written as SCD.
Painful hand and foot swelling (dactylitis) with anaemia is sickle cell disease.
Treatment at a glance
- Folic acid daily
- Penicillin V prophylaxis in young children
- Pneumococcal and other vaccines
Sickle Cell Disease (×2)
- Q: What is shown on the blood smear?
- Sickle Cell Disease (sickle cell anaemia)
- Q: Describe the abnormal findings?
- Numerous RBCs with crescent/sickle shape
- Contrast to normal biconcave disc-shaped RBCs
Read this in EXAM: Spot/Practical Examination
What our notes say
- Painful hand and foot swelling (dactylitis) with anaemia is sickle cell disease. (Paediatrics Senior Clerkship end of 2nd semester exam: 4 October 2021, 50 MCQs)
- Children with sickle cell disease are susceptible to pneumococcal infection (Paediatrics end of fourth year special supplementary exam: August 2019, Paper On)
- Sickle cell disease is inherited in an X-linked recessive pattern (Paediatrics end of fourth year special supplementary exam: August 2019, Paper On)
- Children with sickle cell disease are functionally asplenic and at high risk of overwhelming sepsis from encapsulated organisms, so fever is an emergency. (Paediatrics Question Bank: 12 Clinical MCQs)
- There is enough fetal hemoglobin at birth and through infancy to prevent significant sickling with sickle cell disease. (hematopathology — Past Paper Questions & Answers)
- Topics tested: respiratory disease, sickle cell disease and anaemias, rheumatic fever, diabetes, TB, pulmonary embolism, connective tissue disease, HIV, gastroenterology, thyroid disease, gout, leukaemia and lymphoma, kidney disease and stroke. (Internal Medicine (MBIM 4100) main exam, Paper One: 3 July 2019)
Study material on Sickle Cell Disease
- Sickle Cell Disease: Pathology, Genetics & Mechanisms
- GENETIC DISORDERS SAQs EXAMINATION
- Paediatrics Senior Clerkship end of 2nd semester exam: 4 October 2021, 50 MCQs
- Paediatrics Question Bank: 12 Clinical MCQs
- Paediatrics end of fourth year special supplementary exam: August 2019, Paper One, 87 of 100 MCQs
- Approach to anaemia
- Pathology Practical Revision Guide
- Hematopathology MCQs & Essays: MBChB Exam Revision Guide
Practice questions on Sickle Cell Disease
NOT a typical feature of sickle cell disease?
- Vaso-occlusive crises
- Aplastic crises
- Splenic sequestration
- Hemolytic anemia
- Microcytosis
Answer: A. SCD is a normocytic hemolytic anemia.* ---
In sickle cell disease, sickling is promoted by all of the following EXCEPT
- Hypoxia
- Dehydration
- High altitude
- High fetal haemoglobin
Answer: D. HbF inhibits HbS polymerization by disrupting the deoxygenated HbS polymer structure; high HbF is protective, not a trigger. ---
Which is NOT a typical feature of sickle cell disease?
- Vaso-occlusive crises
- Aplastic crises
- Splenic sequestration
- Hemolytic anemia
- Microcytosis
Answer: E. Sickle cell disease produces normocytic anemia; microcytosis is not a feature unless co-existing iron deficiency is present. ---