Hereditary Spherocytosis
Also written as HS.
Most cases are asymptomatic and do not require\nspecific treatment; more severe cases are managed in a similar way\nto hereditary spherocytosis.
What our notes say
- Most cases are asymptomatic and do not require\nspecific treatment; more severe cases are managed in a similar way\nto hereditary spherocytosis. (Davidson's Essentials of Medicine - 2nd)
- The RBC life span is much shorter in patients with hemolysis secondary to conditions such as hereditary spherocytosis, immune hemolytic anemia, or sickle cell disease.\n (Anemia of Inadequate production)
- Hereditary spherocytosis (HS) is a common cause of inherited hemolytic anemia, with a prevalence of approximately 1 in 2,000-5,000 persons.\n (Haemolytic Anaemias)
- Hereditary spherocytosis is usually transmitted as an autosomal dominant trait.\n (Haemolytic Anaemias)
- The red cell defects such as hereditary spherocytosis pregnancy and is monitored by ultrasound scans, cerebral (Devendra K. Gupta, Krishna M. Goel - Hutchison’s Paediatrics. chdhe-Jaypee Broth)
- terminal ileum there is a risk of a megaloblastic anaemia Hereditary Spherocytosis (Congenital (Devendra K. Gupta, Krishna M. Goel - Hutchison’s Paediatrics. chdhe-Jaypee Broth)
Study material on Hereditary Spherocytosis
- Haemolytic anaemia
- Devendra K. Gupta, Krishna M. Goel - Hutchison’s Paediatrics. chdhe-Jaypee Brothers (2012)
- Haematology MCQs
- Here are all 45 questions, complete with full stems, choices, and answers. — Study Notes
- Hematopathology
- Haemolytic Anaemias
- Haemolytic Anaemias
- Haematopathology MCQs: Blood Disorders & Anemia Explained — MCQ Bank
- Hematopathology MCQs & Essays: MBChB Exam Revision Guide — Revision Guide
- Davidson's Essentials of Medicine - 2nd
Practice questions on Hereditary Spherocytosis
Most common cause of hereditary spherocytosis?
- Spectrin deficiency
- Band 3 deficiency
- Protein 4.1 deficiency
- Ankyrin deficiency
- Glycophorin A deficiency
Answer: D. Ankyrin defects (isolated or combined with spectrin) are the most frequent cause.
Hereditary spherocytosis is most commonly due to deficiency of
- Pyruvate kinase
- G6PD
- Band 3 protein only
- Spectrin or ankyrin
Answer: D. Spectrin/ankyrin deficiency destabilizes the RBC membrane skeleton, causing membrane loss and spherocyte formation with reduced osmotic resistance.