Table of Contents
Definition / general | Diagrams / tables | Treatment | Gross description | Microscopic (histologic) description | Peripheral smear imagesCite this page: Mansouri J. Hereditary spherocytosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenHS.html. Accessed December 2nd, 2024.
Definition / general
- Congenital hemolytic anemia due to genetically determined abnormal spectrin and ankyrin molecules, leading to defects in red blood cell membrane, causing spherical shape and lack of plasticity
- Red blood cells become trapped within spleen and have less than usual 120 day lifespan
- Splenic function is normal
- Osmotic fragility: increased; basis for diagnostic testing
Diagrams / tables
Images hosted on other servers:
Scatter diagram of CBC
Treatment
- Splenectomy (prolongs survival of red blood cells, although they still have membrane defects)
Gross description
- Firm, deep red tissue, thin capsule, no grossly identifiable Malpighian follicles, 100 - 1000 g
Microscopic (histologic) description
- Marked congestion in cords
- Sinuses appear empty but actually contain ghost red blood cells
- May have prominent endothelial lined sinuses, hemosiderin deposition, erythrophagocytosis
Peripheral smear images
Images hosted on other servers:
Small spherocytes
