NDT Advance Access originally published online on February 1, 2007
Nephrology Dialysis Transplantation 2007 22(5):1471-1472; doi:10.1093/ndt/gfm038
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
(Section Editor: G. H. Neild)
Lingual amyloidosis—a rare complication of long-term haemodialysis
1Kidney Institute and Department of Nephrology, 2Department of Hematology, 3Department of Pathology, Chang Gung Memorial Hospital, Chang Gung University, Taipei, Taiwan and 4Department of Medicine D, Division of General Internal Medicine and Nephrology, University Hospital Muenster, Muenster, Germany
Correspondence and offprint requests to: Hsiang-Hao Hsu, MD, Kidney Institute and Department of Nephrology, Chang Gung Memorial Hospital, Chang Gung University, 199 Tun-Hwa North Road, Taipei 105, Taiwan. Email: hsianghao{at}adm.cgmh.org.tw
Keywords: ß2 microglobulin; bio-incompatible dialyzer; lingual dysfunction; tongue
Case
A 54 year-old male was diagnosed with polycystic kidney disease in uraemic stage, and had been dialysed for 25 years, since the age of 29 years. He received haemodialysis by artificial kidneys with unsubstituted cellulose membrane for the first 16 years. Carpal tunnel syndrome (CTS) developed soon after, and this patient received surgical intervention over bilateral wrists in the 8th year of maintenance dialysis. He was also diagnosed radiologically as suffering from destructive spondyloarthropathy (DSA) since the 13th year, with unsubstituted cellulose membrane dialysers. He was shifted to modified cellulose membrane dialysers in his last 9-year period of dialysis therapy.
He was first noticed to have multiple small, pale-yellowish nodules on the dorsal and lateral surface of tongue several years ago, initially without lingual motor dysfunction. These lingual nodules enlarged gradually, and also spread extensively to occupy the entire surface of the patient's tongue (Figure 1). This resulted in difficulty in swallowing, loss of taste and slurred speech to the patient. An excision biopsy was performed on these tongue nodules. Basing on the histology findings (positive Congo red stained amyloid depositions, shown in Figure 2), the lack of serum proteins suggestive of a monoclonal gammopathy and the absence of recognizable causes for amyloid A amyloidosis by extensive history review, this patient's diagnosis is presumed to be dialysis-related amyloidosis (DRA).
|
|
The patient's artificial kidney was further changed to high-flux polysulfone dialysers under the clinical impression of DRA. However, he developed malnutrition progressing to cachexia, and died of pneumonia months later.
Discussion
DRA is caused by tissue deposition of ß2-microglobulin as amyloid fibrils in long-term dialysis patients [1], and the occurrence of DRA is closely linked to the duration of dialysis and the use of low-flux, bio-incompatible cellulose membrane [2]. DRA predominantly involves the osteoarticular system, and manifests clinically as CTS, bone cysts, DSA, pathological fractures and scapulohumeral periarthritis. As the number of long-term dialysis patients has increased, DRA have been found occasionally to involve gastrointestinal, cardiac, pulmonary and cutaneous system.
DRA of the tongue is a rare and late complication, which occurs in patients undergoing chronic haemodialysis for >20 years [3]. The lingual amyloid nodules were firm in consistency, whitish-yellow in colour and varied with sizes of >1 mm in diameter. Advanced DRA of the tongue leads to lingual dysfunction, such as abnormal taste, altered mobility and articulatory imprecision. With severe and prolonged lingual dysfunction, malnutrition and other associated comorbidity can occur. Altering the duration, frequency and type of dialysis treatment with biocompatible high flux dialysers are potentially helpful strategies to prevent DRA-related morbidity.
Conflict of interest statement. None declared.
References
- Gejyo F, Yamada T, Odani S, et al. (1985) A new form of amyloid protein associated with chronic hemodialysis was identified as beta 2-microglobulin. Biochem Biophys Res Commun 129:701–706.[CrossRef][Web of Science][Medline]
- Schiffl H, Fischer R, Lang SM, Mangel E. (2000) Clinical manifestations of AB-amyloidosis: effects of biocompatibility and flux. Nephrol Dial Transplant 15:840–845.
[Abstract/Free Full Text] - Matsuo K, Nakamoto M, Yasunaga C, Goya T, Sugimachi K. (1997) Dialysis-related amyloidosis of the tongue in long-term hemodialysis patients. Kidney Int 52:832–838.[Web of Science][Medline]
Accepted in revised form: 11. 1.07
CiteULike 
Connotea 
Del.icio.us What's this?
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||