Spleen
Other nonneoplastic disorders
Hereditary spherocytosis

Author: Jaleh Mansouri, M.D. (see Authors page)

Revised: 2 March 2018, last major update October 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Hereditary spherocytosis[TI] spleen

Cite this page: Mansouri, J. Hereditary spherocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenHS.html. Accessed August 20th, 2018.
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
Microscopic (histologic) images

Images hosted on other servers:

Marked congestion in splenic cords

Peripheral smear images

Images hosted on other servers:

Small spherocytes