Summary
A 73-year-old man with chronic renal failure of undetermined aetiology had received haemodialysis for 12 years when he died of acute purulent peritonitis due to caecal perforation. Amyloid deposits detected in a cystic bone lesion in the left hip had caused a pathological fracture 17 days before death. At autopsy, extensive amyloid deposits were found in the osteoarticular system, in the cartilaginous surface and the capsular tissue of joints, ligaments, vertebral discs and bone. In addition, vascular amyloid deposits were diagnosed in the heart, kidneys, testes, lungs, skin and in the gastrointestinal tract. A special feature of this case were interstitial amyloid deposits forming a fine-meshed structure in the myocardium and plate-like deposits in the gastrointestinal tract. Immunohistochemically, all these deposits reacted strongly with antibody to humanβ 2-microglobulin but showed no reaction with antibodies to AA, Alambda, A-kappa and AF. The present case demonstrates that extra-osteoarticular manifestations of AB-amyloidosis can cause serious complications.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Allain TJ, Stevens PE, Bridges LR, Phillips ME (1988) Dialysis myelopathy: quadriparesis due to extradural amyloid ofβ2 microglobulin origin. BMJ 296:752–753
Assenat H, Calemard E, Charra B, Laurent G, Terrat JC, Vanel T (1980) Hemodialyse syndrome du canal carpien et substance amyloide. Nouv Presse Méd 9:1715
Bardin T, Kuntz D, Zingraff J, Voisin MC, Zelmar A, Lansaman J (1985) Synovial amyloidosis in patients undergoing long-term hemodialysis. Arthritis Rheum 28:1052–1058
Bindi P, Chanard J (1990) Destructive spondylarthropathy in dialysis patients: an overview. Nephron 55:104–109
Campistol JM, Sole M, Munoz-Gomez J, Riba J, Ramon R, Revert L (1990) Patholgoical fracture in patients who hve amyloidosis associated with dialysis. J Bone Joint Surg 72:568–574
Choi HSH, Heller D, Picken MM, Sidhu GS, Kahn T (1989) Infarction of intestine with massive amyloid deposition in two patients on long-term hemodialysis. Gastroenterology 96:230–234
Clanet M, Mansat M, Durroux R, Testut MF, Guiraud B, Rascol A, Conte J (1981) Syndrome du canal carpien, tenosynovite amyloide et hemodialyse periodique. Rev Neurol (Paris) 137:613–624
Floege J, Brandis A, Nonnast-Daniel B, Westhoff-Bleck M, Tiedow G, Linke RP, Koch KM (1989) Subcutaneous amyloid-tumor of beta-2-microglobulin origin in a long-term hemodialysis patient. Nephron 53:73–75
Fuchs A, Jagirdar J, Schwartz IS (1987) Beta 2-microglobulin amyloidosis (AB2M) in patients undergoing long-term hemodialysis. Am J Clin Pathol 88:302–307
Gejyo F, Yamada T, Odani S, Nakagawa Y, Arakawa M, Kunito-mo T, Katauka H, Suzuki M, Hirasawa Y, Shirahama T, Cohen AS, Schmid K (1985) A new form of amyloid protein associated with chronic hemodialysis was identified as beta-2-microglobulin. Biochem Biophys Res Commun 129:701–706
Goldstein S, Winston E, Chung TJ, Chopra S, Pariser K (1985) Chronic arthropathy in long-term hemodialysis. Am J Med 78:82–86
Guccion JG, Redman RS, Winne CE (1989) Hemodialysis-associated amyloidosis presenting as lingual nodules. Oral Surg Oral Med Oral Pathol 68:618–623
Huaux JP, Noel H, Bastien P, Malghem J (1985) Amylose articulaire, fracture du col femoral et hemodialyse periodique chronique. Rev Rhum 52:179–182
Kuntz D, Naveau B, Bardin T, Drüeke T, Treves R, Dryll A (1984) Destructive sponylarthropathy in hemodialyzed patients. A new syndrome. Arthritis Rheum 27:369–375
Linke RP, Bommer J, Ritz E, Waldherr R, Eulitz M (1986) Amyloid kidney stones of uremic patients consist of beta-2-microglobulin fragments. Biochem Biophys Res Commun 136:665–671
Maher ER, Hamilton-Dutoit S, Baillod RA, Sweny P, Moorhead JF (1988) Gastrointestinal complications of dialysis related amyloidosis. BMJ 297:265–266
Mohr W (1984) Arthrosis deformans. In: Doerr W, Seifert G (eds) Spezielle pathologische Anatomie, vol 18/I. Springer, Berlin Heidelberg New York, pp 257–372
Munoz-Gomez J, Bergada-Barado E, Gomez-Perez R, Llopart-Buisan E, Subias-Sobrevia E, Rotes-Querol J, Sole-Arques M (1985) Amyloid arthropathy in patients undergoing periodic hemodialysis for chronic renal failure: a new complication. Ann Rheum Dis 44:729–733
Noel LH, Zingraff J, Bardin T, Atienza C, Kuntz D, Drüeke T (1987) Tissue distribution of dialysis amyloidosis. Clin Nephrol 27:175–178
Ogawa H, Saito A, Hirabayashi N, Hara K (1987) Amyloid deposition in systemic organs in long-term hemodialysis patients. Clin Nephrol 28:199–204
Ozasa H, Suzuki T, Takahashi K, Ota K (1989) Protein components of amyloid-like kidney stones of chronic hemodialysis patients. Nephron 53:257–260
Puchtler H, Sweat F, Levine M (1962) On the binding of congo red by amyloid. J Histochem Cytochem 10:355–364
Scheumann GFW, Nerlich ML, Reilmann H, Wittekind C (1989) Pathologische Frakturen bei dialyseassoziierter Amyloidose. Unfallchirurg 92:68–72
Schober R, Passlick J, Kiwit JCW, Frenzel H, Linke RP (1990) Destructive Spondylopathie beiβ2-Mikroglobulin-bedingter Amyloidose. Pathologe 11:154–157
Sebert J, Fardellone P, Marie A (1986) Destructive spondyl-arthropathy in hemodialyzed patients: possible role of amyloidosis. Arthritis Rheum 29:301–302
Sethi D, Hutchison AJ, Cary NRB, Brown EA, Curtis JR, Wood-row DF, Gower PE (1990) Macroglossia and amyloidoma of the buttock: evidence of systemic involvement in dialysis amyloid. Nephron 55:312–315
Shinoda T, Komatsu M, Aizawa T, Shirota T, Yamada T, Ehara T (1989) Intestinal pseudo-obstruction due to dialysis amyloidosis. Clin Nephrol 32:284–289
Takahashi S, Morita T, Koda Y, Murayama H, Hirasawa Y (1988) Gastrointestinal involvement of dialysis-related amyloidosis. Clin Nephrol 30:168–171
Theaker JM, Raine AEG, Rainey AJ, Heryet A, Clark A (1987) Systemic amyloidosis ofβ2-microglobulin type: a complication of long term haemodialysis. J Clin Pathol 40:1247–1251
Warren DJ, Otieno LS (1975) Carpal tunnel syndrome in patients on intermittent hemodialysis. Postgrad Med J 51:450–454
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Zhou, H., Pfeifer, U. & Linke, R. Generalized amyloidosis from β2-microglobulin, with caecal perforation after long-term haemodialysis. Vichows Archiv A Pathol Anat 419, 349–353 (1991). https://doi.org/10.1007/BF01606526
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01606526