Al amyloidosis renal changesE85.9
Synonym(s)
DefinitionThis section has been translated automatically.
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.
Occurrence/EpidemiologyThis section has been translated automatically.
(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.
Clinical featuresThis section has been translated automatically.
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
TherapyThis section has been translated automatically.
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)
Progression/forecastThis section has been translated automatically.
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
LiteratureThis section has been translated automatically.
- Favà A et al (2018) Treatment of multiple myeloma with renal involvement: the nephrologist's view. Clin Kidney J 11:777-785.
- Huang X et al (2016) Bortezomib with dexamethasone as first-line treatment for AL amyloidosis withrenal involvement. Amyloid 23:51-57.
- 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.
- Kanzaki G et al (2019) Monoclonal Immunoglobulin Deposition Disease and Related Diseases. J Nippon Med Sch 86:2-9.
- Leung N et al (2018) Dysproteinemias and Glomerular Disease. Clin J Am Soc Nephrol 13:128-139.
- Sallée M et al (2019) Myeloma cast nephropathy: the dusk of high cutoff haemodialysis. Lancet Haematol 6:e174-e176.