[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia].
Waldenström's macroglobulinemia (W.M.) is a paraproteinemic disorder similar to multiple myeloma expressing a monoclonal IgM paraprotein. The course of W.M. is milder and the occurrence of renal disease is less frequent than in multiple myeloma. Amyloidosis occurs in less than 5% of patients with monoclonal IgM. We report a 65 year old patient with an asymptomatic long-term survival whose W.M. remained without any therapy. Amyloidosis developed twenty-one years after the initial diagnosis presenting with the nephrotic syndrome, renal and heart failure. W.M. combined with amyloidosis is a rare association. The deposition of amyloid causes progressive organ damage and indicates a bad prognosis. Most commonly nephrotic syndrome or heart failure are signs of amyloidosis. The development of amyloidosis does not seem to be influenced by the duration of W.M. disease or the paraprotein level. It is not known whether the amyloidosis can be prevented by an early chemotherapy.
Zimmermann
I
I
Medizinische Abteilung, Kantonsspital Schaffhausen.
Gloor
H J
HJ
Rüttimann
S
S
D000368:Aged; D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000209:etiology* / Q000473:pathology; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology / Q000473:pathology; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007668:Kidney / Q000473:pathology; D008297:Male; D009206:Myocardium / Q000473:pathology; D009404:Nephrotic Syndrome / Q000209:etiology / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000473:pathology
D000682:Amyloid; C035222:amyloid protein AL
Generalisierte AL-Amyloidose: Eine seltene Komplikation bei Makroglobulinämie Waldenström.