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Diagnose und Therapie der Nierenbeteiligung bei Plasmazellerkrankungen

Renale Beteiligung bei multiplem Myelom und monoklonalen Gammopathien

Diagnosis and treatment of kidney involvement in plasma cell diseases

Renal involvement in multiple myeloma and monoclonal gammopathies

  • Schwerpunkt: Multiples Myelom
  • Published:
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Zusammenfassung

Hintergrund

Eine Nierenbeteiligung ist eine häufige und prognostisch relevante Organmanifestation bei Plasmazellerkrankungen.

Fragestellung

Darstellung des Spektrums der renalen Beteiligung bei Plasmazellerkrankungen sowie der diagnostischen und therapeutischen Empfehlungen.

Material und Methoden

Evaluation und Diskussion der aktuellen Fachliteratur sowie der Empfehlungen der Fachgesellschaften.

Ergebnisse

Die klinischen Manifestationsformen einer renalen Beteiligung bei Plasmazellerkrankungen sind heterogen und reichen von der akuten Cast-Nephropathie beim multiplen Myelom bis hin zu seltenen Glomerulonephritisformen. Eine Nierenbeteiligung im Rahmen einer monoklonalen Gammopathie ohne Hinweis auf ein multiples Myelom wird als monoklonale Gammopathie mit renaler Signifikanz bezeichnet. Häufig findet sich eine renale Ablagerung monoklonaler Immunglobuline („monoclonal immunoglobulin deposition disease“ [MIDD]) oder eine renale Leichtketten(AL)-Amyloidose. Entscheidend ist die weiterführende hämatologische Diagnostik, um das Vorliegen einer hämatologischen Neoplasie auszuschließen. Die Therapie einer renalen Beteiligung bei monoklonalen Gammopathien besteht in einer Verminderung des Plasmazellklons mittels zytoreduktiver Therapie. Die Reduktion der monoklonalen Proteine im Serum ist für das renale Therapieansprechen prognostisch relevant. Bei histologischem Nachweis einer Cast-Nephropathie wird zudem eine Reduktion der freien Leichtketten mittels High-cut-off-Dialyse empfohlen.

Diskussion

Das Spektrum der renalen Manifestation bei Plasmazellerkrankungen hat sich insbesondere seit Einführung des Begriffs der monoklonalen Gammopathie mit renaler Signifikanz erweitert und bleibt eine interdisziplinäre Herausforderung.

Abstract

Background

Kidney involvement is a common complication in patients with plasma cell diseases.

Objective

This article outlines the spectrum of renal involvement in plasma cell dyscrasia and describes diagnostic and therapeutic measures to guide clinical management.

Material and methods

Evaluation and discussion of the current literature as well as existing guidelines and recommendations of professional societies.

Results

The clinical manifestations of renal involvement in plasma cell disorders are heterogeneous and range from acute cast nephropathy in multiple myeloma to rare forms of glomerulonephritis. The term monoclonal gammopathy of renal significance (MGRS) was introduced to describe kidney involvement caused by monoclonal gammopathy but without evidence for underlying malignancy. Light chain cast nephropathy is the most common renal manifestation in multiple myeloma, whereas monoclonal immunoglobulin deposition disease (MIDD) and renal light chain (AL) amyloidosis can be found in multiple myeloma and MGRS. Decisive is the extended hematological diagnostics in order to exclude the presence of a hematological neoplasm. The treatment of renal involvement in monoclonal gammopathies involves the reduction of the plasma cell clone with cytoreductive treatment. The reduction of the monoclonal protein in serum is prognostically relevant for the renal response to treatment. In the case of histological evidence of a light chain cast nephropathy, high cut-off dialysis is recommended to reduce the free light chains in serum.

Conclusion

The spectrum of renal manifestations in plasma cell dyscrasia has been expanded, particularly since the introduction of the term MGRS. Diagnostic and therapeutic management remain an interdisciplinary challenge.

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Abbreviations

ACE:

„Angiotensin-converting enzyme“

AH:

Heavy-chain-Amyloidose

AHL:

Heavy-and-light-chain-Amyloidose

AIg:

Immunglobulinassoziierte Amyloidose

AL:

Light-chain- bzw. Leichtkettenamyloidose

ASFA:

American Society for Apheresis

C3:

Komplementfaktor 3

CRAB-SLiM:

Diagnosekriterien des multiplen Myeloms („calcium, renal failure, anemia, bone disease, ≥60 % bone marrow plasma cells, light chain ratio ≥100, focal bone lesion MRI“)

eGFR:

Geschätzte („estimated“) glomeruläre Filtrationsrate

FLC:

„Free light chains“

GN:

Glomerulonephritis

HCDD:

„Heavy chain deposition disease“

HD:

Hämodialyse

HDF:

Hämodiafiltration

HE:

Hämatoxylin-Eosin

HCO:

„High cut-off“

HF:

„High flux“

Ig:

Immunglobulin

kDA:

Kilodalton

LCDD:

„Light chain deposition disease“

LK:

Leichtketten

MGRS:

Monoklonale Gammopathie mit renaler Signifikanz

MGUS:

Monoklonale Gammopathie unbestimmter Signifikanz

MIDD:

„Monoclonal immunoglobulin deposition disease“

MG:

Monoklonale Gammopathie

MM:

Multiples Myelom

NSAID:

Nichtsteroidale Antiphlogistika

PAS:

„Periodic acid Schiff“

PE:

Plasmapherese

PGNMID:

Proliferative Glomerulonephritis mit monoklonalen Immunglobulinablagerungen

POEMS:

„Polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes“

RANKL:

„Receptor activator of NF-κB ligand“

TMA:

Thrombotische Mikroangiopathie

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Authors and Affiliations

  1. Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    V. Gödecke,  J. J. Schmidt & Haller H.

  2. Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    J. H. Bräsen

  3. Klinik für Hämatologie, Hämostaseologie, Onkologie und Stammzelltransplantation, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    C. Koenecke

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Correspondence to V. Gödecke.

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Interessenkonflikt

C. Koenecke: Beratungstätigkeit für AbbVie, Amgen, BMS, Roche. V. Gödecke, J.J. Schmidt, J.H. Bräsen und H. Haller geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Gödecke, V., Schmidt, J.J., Bräsen, J.H. et al. Diagnose und Therapie der Nierenbeteiligung bei Plasmazellerkrankungen. Internist 60, 10–22 (2019). https://doi.org/10.1007/s00108-018-0538-7

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