Lymph nodes & spleen, nonlymphoma

Spleen-other nonneoplastic disorders

Hereditary spherocytosis

Last author update: 1 October 2012
Last staff update: 12 October 2020

Copyright: 2003-2024,, Inc.

PubMed Search: Hereditary spherocytosis[TI] spleen

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Cite this page: Mansouri J. Hereditary spherocytosis. website. Accessed May 19th, 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

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Scatter diagram of CBC

  • 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

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Small spherocytes

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