Alpha-mannosidosis. Report of a case with morphologic, cytologic and immunohistochemical considerations

Acta Cytol. 1994 May-Jun;38(3):441-5.


We report the histopathologic features of the knee bone and synovium and the cytologic features of the synovial fluid from a patient with alpha-mannosidosis. The synovium showed marked papillary hyperplasia with infiltration of foamy histiocytes containing periodic acid-Schiff-positive, diastase-resistant material. Severe degenerative changes were seen in the knee bone. The synovial fluid showed increased numbers of macrophages containing periodic acid-Schiff-positive, diastase-resistant material. The differential diagnostic considerations in the synovial fluid are also discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Erythrocytes / pathology
  • Humans
  • Hyperplasia
  • Knee Joint / pathology*
  • Knee Joint / surgery
  • Macrophages / pathology
  • Male
  • Mannosidases / deficiency*
  • Synovial Fluid / cytology*
  • Synovial Membrane / pathology*
  • Synovial Membrane / ultrastructure
  • Vacuoles / pathology
  • Vacuoles / ultrastructure
  • alpha-Mannosidase
  • alpha-Mannosidosis / pathology*


  • Mannosidases
  • alpha-Mannosidase