Al amyloidosis renal changes E85.9

AL-amyloidosis of the kidney (see also systemic amyloidosis) is defined by fibrillary deposits of amyloid in the renal parenchyma, consisting of monoclonal light chains.

(Nephrogenic) AL amyloidosis occurs not only in multiple myeloma but also in monoclonal gammopathy of indeterminate significance (MGUS). In about 30% of patients with AL amyloidosis, multiple myeloma can be diagnosed as the underlying disease. 15% of patients with multiple myeloma develop AL amyloidosis with renal involvement.

In systemic AL amyloidosis, 1 or more organ involvement occurs:

• renal involvement (in 50%): nephrotic syndrome
• restrictive cardiomyopathy (at 30%)
• AL skin and mucous membrane changes (30-50%): Purpura (face, especially eyelids, scalp, palmae and plantae), less frequently nodular skin changes.
• Liver involvement (16%)
• Amyloid arthropathy (especially in beta2-micoglobulin-associated amyloidosis)
• 50% show a reduced glomerular filtration rate at diagnosis
• Hypertension (25%)
• peripheral neuropathymacroglossia
• Carpal tunnel syndrome

Oral combination therapy with melphalan and prednisolone improves survival rate for a few months

High-dose chemotherapy with melphalan and autologous stem cell transplantation

Bortezomib with dexamethasone (Huang X et al. 2016)

The AL - amyloidosis has a bad prognosis if left untreated:

• the average survival time is 12 months
• cardiac involvement leads to death in 40% of patients

1. Favà A et al (2018) Treatment of multiple myeloma with renal involvement: the nephrologist's view. Clin Kidney J 11:777-785.
2. Huang X et al (2016) Bortezomib with dexamethasone as first-line treatment for AL amyloidosis withrenal involvement. Amyloid 23:51-57.
3. Hutchison CA et al. (2019) High cutoff versus high-flux haemodialysis for myeloma cast nephropathy inpatients receiving bortezomib-based chemotherapy (EuLITE): a phase 2 randomised controlled trial.Lancet Haematol 6:e217-e228.
4. Kanzaki G et al (2019) Monoclonal Immunoglobulin Deposition Disease and Related Diseases. J Nippon Med Sch 86:2-9.
5. Leung N et al (2018) Dysproteinemias and Glomerular Disease. Clin J Am Soc Nephrol 13:128-139.
6. Sallée M et al (2019) Myeloma cast nephropathy: the dusk of high cutoff haemodialysis. Lancet Haematol 6:e174-e176.