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Abstract: SA-PO1277

Renal Manifestations in Patients Diagnosed with Waldenstrom Macroglobulinemia

Session Information

Category: Onconephrology

  • 1600 Onconephrology

Authors

  • Fine, Rachel L., UK HealthCare, Lexington, Kentucky, United States
  • Finke, Ann R., UK HealthCare, Lexington, Kentucky, United States
  • Ayach, Taha, UK HealthCare, Lexington, Kentucky, United States
  • Alagusundaramoorthy, Sayee Sundar, UK HealthCare, Lexington, Kentucky, United States
Introduction

Waldenstrom’s macroglobulinemia (WM) is a rare B-cell lymphoma in which abnormal B-cells produce high levels of IgM paraprotein and infiltrate the bone marrow and other organs of the lymphatic system. WM has been shown to cause some degree of renal dysfunction in up to 5% of affected patients. Herein, we present two cases of unusual renal manifestations in the setting of WM, papillary necrosis and Fanconi’s syndrome.

Case Description

Case 1: 69-year-old female without prior medical history presented for evaluation of episodic gross hematuria. Initial U/A >50 RBCs/HPF with mild left hydroureteronephrosis on CT urogram. Subsequent cystoscopy, bilateral ureteroscopy, and left retrograde pyelogram showed bleeding from the left ureteral orifice and renal papilla with left renal papillary necrosis without evidence of upper tract tumor. Labs showed a normocytic anemia (Hgb 8.7), elevated serum IgM (>5850), SPEP with monoclonal gamma protein spike, KLC 17.82, LLC 104.27, and K/L ratio 0.17. BM bx revealed hypercellular marrow with 30-40% plasmacytic differentiation suggestive of WM. Given the concern for hyperviscosity syndrome, plasma exchange was instituted while directed treatment with rituximab was begun.
Case 2: 64 y.o. male with T2DM and HTN referred to BMT clinic for elevated IgM Kappa M protein. BM bx demonstrated >10% lymphoplasmacytic cells with a MYD88 mutation. Over the next year, the patient developed worsening renal function with sCr 2.7 (baseline 1.6), persistent anemia, glucosuria (3+), proteinuria (3+), generalized amino aciduria, and a non-gap metabolic acidosis. UPEP revealed elevated IgM Kappa Bence Jones protein. Renal bx demonstrated IgM mesangial/paramesangial immune complex deposition. Diagnosis of Fanconi’s syndrome in the setting of WM was made and BTK inhibitor therapy initiated.

Discussion

WM often takes an indolent course presenting with non-specific symptoms with rare renal involvement. Review of the current literature shows an association with amyloidosis, cryoglobulinemia, hyperviscosity syndrome, and hemolytic anemia. Renal dysfunction in these patients remains an important consideration as kidney involvement is present in 3-5% of patients. These cases demonstrate the significant and disparate effects of WM on the renal system along with the importance of a multi-disciplinary approach in the diagnosis and management of this disease.