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. 2024 Jun 19;17(8):sfae185.
doi: 10.1093/ckj/sfae185. eCollection 2024 Aug.

Kidney involvement in myelodysplastic syndromes

Marie-Camille Lafargue  1 , Jean-Paul Duong Van Huyen  2 , Helmut G Rennke  3 , Marie Essig  4 , Mickaël Bobot  5 , Noémie Jourde-Chiche  5 , Hamza Sakhi  6 , Alexandre Karras  7 , Idris Boudhabhay  6 , Philippe Brunet  5 , Hugoline Boulay  8 , Vincent Grobost  9 , Carole Philipponnet  10 , Juliette Jeannel  11 , Jonathan Chemouny  12 , Jean-Jacques Boffa  13 , Dorra Braham-Stambouli  14 , Umut Selamet  15 , Leonardo V Riella  16 , Olivier Fain  17 , Lionel Adès  18 , Pierre Fenaux  18 , Camille Cohen  19 , Arsène Mekinian  17
Affiliations

Kidney involvement in myelodysplastic syndromes

Marie-Camille Lafargue et al. Clin Kidney J. .

Abstract

Introduction: The objective of this study was to describe kidney involvement in patients with myelodysplastic syndromes (MDS), their treatments, and outcomes.

Methods: We conducted a multicenter retrospective study in seven centers, identifying MDS patients with acute kidney injury (AKI), chronic kidney disease (CKD), and urine abnormalities.

Results: Fifteen patients developed a kidney disease 3 months after MDS diagnosis. Median urine protein-to-creatinine ratio was 1.9 g/g, and median serum creatinine was 3.2 mg/dL. Ten patients had AKI at presentation, and 12 had extra-renal symptoms. The renal diagnoses included anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), ANCA negative vasculitis, C3 glomerulonephritis, immune complex-mediated glomerulonephritis, polyarteritis nodosa, and IgA vasculitis. All patients but one received a specific treatment for the MDS-associated kidney injury. The effect of MDS treatment on kidney injury could be assessed in six patients treated with azacitidine, and renal function evolution was heterogenous. After a median follow-up of 14 months, four patients had CKD stage 3, five had CKD stage 4, and three had end stage kidney disease. On the other hand, three evolved to an acute myeloid leukemia and three died. Compared to 84 MDS controls, patients who had kidney involvement were younger, had a higher number of dysplasia lineages, and were more eligible to receive hypomethylating agents, but no survival difference was seen between the two groups. Compared to 265 AAV without MDS, the ten with MDS-associated pauci-immune vasculitis were older, ANCA serology was more frequently negative, and more cutaneous lesions were seen.

Conclusion: The spectrum of kidney injuries associated with MDS is mostly represented by vasculitis with glomerular involvement, and especially AAV.

Keywords: ANCA-associated vasculitis; acute kidney injury; auto-immunity; myelodysplastic syndromes; myeloid neoplasms.

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Conflict of interest statement

A.M. is investigator of CELGENE, ROCHE, CHUGAI funded trials with APHP, and Hôpital 15-20 promotion; A.M. received several fees for congress travels and expert use from LFB, SANOFI, SHIRE, and CELGENE.

Figures

Figure 1:
Figure 1:
Kidney biopsy of a 28-year-old man with a history of mono-MAC syndrome (monocytopenia and mycobacterial infection syndrome) and MDS referred to renal clinic for evaluation of a nephrotic syndrome with hematuria. Focal proliferative and crescentic glomerulonephritis, pauci-immune cellular crescents and fibrocellular or fibrous crescents with segmental scarring (hematoxylin and eosin staining, magnification ×177). Courtesy of Helmut G Rennke.
Figure 2:
Figure 2:
(a) Kaplan–Meier curve for the overall survival according to AAV with and without MDS. AAV with MDS group (n = 10). AAV without MDS group (n = 265). Last follow-up at 5 years. P = .0190, log rank test. (b) Kaplan–Meier curve for the overall survival according to MDS with and without kidney injury. MDS with kidney injury (n = 15). MDS without kidney injury (n = 84). Last follow-up at 5 years. P = .7309, log rank test.
See this image and copyright information in PMC

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