Renale Manifestationen von Leichtkettenerkrankungen – Epidemiologie und Prognose

Zusammenfassung

Hintergrund: Eine eingeschränkte Nierenfunktion bei Leichtketten-assoziierten Erkrankungen wird neben der klassischen Myelomniere (MCN) durch die AL-Amyloidose und die monoklonale Immundepositionserkrankung (MIDD) hervorgerufen.

Patienten und Methoden: In einer monozentrischen retrospektiven Analyse wurden Patienten auf das Vorliegen einer renalen Beteiligung untersucht, bei denen von 1996 bis 2008 ein therapiepflichtiges Multiples Myelom (n = 392), eine AL-Amyloidose (n = 53) oder MIDD (n = 12) diagnostiziert wurde. Die einzelnen Formen wurden hinsichtlich ihres Einflusses auf den Eintritt, den Verlauf der Erkrankung und die Prognose miteinander verglichen.

Ergebnisse: Eine Nierenbeteiligung zum Zeitpunkt der Erstdiagnose eines Multiplen Myeloms, einer AL-Amyloidose oder einer MIDD lag bei 45,5 % vor. Es handelte es sich in 68 % um eine MCN, in 25 um eine AL-Amyloidose und in 6% um eine MIDD; Eine Dialysepflicht bestand in 17 %, 8 % bzw. 50 %. Patienten mit einer MIDD/AL-Amyloidose wurden meist durch eine Hypervolämie symptomatisch. Der Zeitraum zwischen dem Auftreten von Symptomen und Diagnosestellung lag bei AL-Amyloidose bei 52 Wochen. Bei Nierenbeteiligung lag das mediane Überleben bei 17 vs. 77 Monaten bei normaler Nierenfunktion. Die mediane Überlebenszeit bei AL-Amyloidose betrug 12 vs. 21 Monate bei MCN. Eine Stabilisierung der Nierenfunktion durch Chemotherapie gelang nur bei MCN. In der multivariaten Analyse ließ sich u. a. die Niereninsuffizienz als unabhängiger Prognosefaktor (Hazard-Ratio 2,88 [2,06–4,0]) nachweisen. Eine autologe Stammzelltransplantation verbesserte bei Niereninsuffizienz Nierenfunktion und Überlebenszeit.

Folgerung: Eine Niereninsuffizienz bei Multiplem Myelom, AL-Amyloidose bzw. MIDD ist ein unabhängiger Prognosefaktor. Die spezifische Therapie, speziell die autologe Stammzelltransplantation, verbessert die Prognose und kann die Nierenfunktion bei MCN stabilisieren.

Abstract

Background: An impaired renal function in light chain associated disorders may be caused by myeloma cast nephropathy (MCN) but also by AL-amyloidosis (AL-A) and monoclonal immundeposition disease (MIDD).

Patients and Methods: In a monocentric, retrospective analysis, patients suffering from multiple myeloma (MM) (n = 392) requiring medical therapy, AL-A (n = 53) or MIDD (n = 12) diagnosed between 1996 and 2008 were evaluated for renal insufficiency. The different patient cohorts were compared in terms of their clinical course and outcome.

Results: Renal insufficiency in MM-, AL-A- or MIDD-patients at the time of diagnosis was found in 45,5 % of the patients. MCN, AL-A and MIDD were found in 68, 25 and 6 %, respectively. Dialysis dependency was seen in 17 % of MCN, in 8 % of AL-A and in 50 % of MIDD patients. Signs of hypervolemia were the leading symptoms in MIDD/AL-A. The time between the occurence of first symptoms and diagnosis was as long as 52 weeks in patients with AL-A. Patients with renal involvement showed a reduced median survival of 17 compared with 77 months in patients with a normal renal function. Median survival was only 12 months in AL-A compared to 21 months in MCN. Stabilization of renal function after chemotherapy occurred only in MCN. Multivariate Cox regression analysis showed impaired renal function as independent risk factor (Hazard-Ratio 2,88 [2,06–4,0]. In terms of survival and kidney function, autologous stem cell transplantation (ASCT) was beneficial for patients with renal involvement.

Conclusion: Renal insufficiency is an independent risk factor in MM, AL-A and MIDD. Specific therapy, especially ASCT may improve prognosis in patients with renal insufficiency and could stabilize renal function in MCN-patients.

Schlüsselwörter

Niere - multiples Myelom - AL-Amyloidose - Leichtkettendepositionserkrankung

Keywords

kidney - myeloma - al-amyloidosis - monoclonal immun-deposition disease

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