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[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia]
I Zimmermann1, H J Gloor, S Rüttimann
1Medizinische Abteilung, Kantonsspital Schaffhausen.
Abstract:
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.
Insights
Waldenström's macroglobulinemia (WM) is a rare IgM paraprotein disorder. In a rare case, amyloidosis developed 21 years after WM diagnosis, leading to organ failure and poor prognosis.
Area of Science:
- Hematology
- Oncology
- Nephrology
Background:
- Waldenström's macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma characterized by monoclonal IgM paraprotein.
- WM typically follows an indolent course with less frequent renal complications compared to multiple myeloma.
- Amyloidosis is an uncommon complication, occurring in less than 5% of patients with monoclonal IgM.
Observation:
- A 65-year-old patient with asymptomatic WM, untreated for 21 years, developed amyloidosis.
- The patient presented with nephrotic syndrome, renal failure, and heart failure secondary to amyloid deposition.
- This case highlights a rare but severe association between WM and systemic amyloidosis.
Findings:
- Amyloidosis developed two decades after the initial WM diagnosis, irrespective of disease duration or paraprotein levels.
- Amyloid deposition led to progressive organ damage, manifesting as nephrotic syndrome and heart failure.
- The development of amyloidosis in WM indicates a poor prognosis.
Implications:
- The rare occurrence of amyloidosis in WM underscores the need for vigilance in monitoring for systemic complications.
- Early chemotherapy's role in preventing amyloidosis in WM remains unknown and warrants further investigation.
- Understanding the pathogenesis of amyloidosis in WM is crucial for improving patient outcomes and therapeutic strategies.