Spleen
Other nonneoplastic disorders
Hereditary spherocytosis


Topic Completed: 1 October 2012

Revised: 18 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Hereditary spherocytosis[TI] spleen

Jaleh Mansouri, M.D., M.P.H.
Page views in 2018: 1,518
Page views in 2019 to date: 944
Cite this page: Mansouri J. Hereditary spherocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenHS.html. Accessed June 24th, 2019.
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

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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
Microscopic (histologic) images

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Marked congestion in splenic cords

Peripheral smear images

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Small spherocytes

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