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Kidney Week

Abstract: FR-PO673

Crystalglobulinemia in Multiple Myeloma: A Rare Case of Survival and Kidney Recovery

Session Information

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Chou, Angela, St George Hospital, Cabramatta, New South Wales, Australia
  • Vonthethoff, Leon, NSW Heath Pathology St George Hospital, Sydney, New South Wales, Australia
  • Ho, Shir-Jing, St George Hospital, Cabramatta, New South Wales, Australia
  • Badve, Sunil, St George Hospital, Cabramatta, New South Wales, Australia
Introduction

Crystalglobulinemia is a rare complication of monoclonal gammopathy wherein crystallised immunoglobulins deposit in various organs causing occlusive vasculopathy, endothelial damage and thrombosis. The reported mortality in this disease is extremely high.

Case Description

We report a case of a 74-year-old female presenting with polyarthralgia, chest pain, petechial rash and rapidly progressive oliguric acute kidney injury requiring dialysis. The serum creatinine on admission was 368 µmol/L, which peaked at 763 µmol/L on day 5 of admission. Troponin-T was 447ng/L (normal <14 ng/L), peaking at 1,223ng/L 7 days later. There were no ischemic changes on ECG. Serum protein electrophoresis showed IgG kappa paraprotein of 25.7 g/L and κ/λ ratio 21.8 (normal 0.26-1.85). Kidney biopsy revealed crystalline eosinophilic casts in the lumens of medullary tubules. Similar crystalline deposits were present in some interlobular arteries with luminal occlusion. Several glomeruli showed similar mainly crystalline deposits in glomerular capillary loops occluding lumens. Ultrastructure showed widening of subendothelial spaces of glomerular capillary loops with subendothelial flocculent material consistent with a thrombotic microangiopathy. Tubular crystal deposits had an organised parallel linear ultrastucture. Bone marrow biopsy confirmed immunoglobulin G (IgG) κ plasma cell multiple myeloma. This patient was successfully treated with 5 sessions of plasmapheresis and clone reduction chemotherapy with 11 cycles of cyclophosphamide, bortezomib and dexamethasone. This resulted in complete resolution of her symptoms, normalisation of troponin-T, reduction of paraprotein levels to <1 g/L and overall excellent kidney and hematological recovery. In 2 months, she was no longer dialysis dependent and in 32 months her latest creatinine had improved to 100 µmol/L.

Discussion

Crystalglobulinemia is a rare and life-threatening illness that should be suspected in patients with rapidly progressive acute kidney injury and monoclonal gammopathy. It can mimic other clinical entities, such as acute coronary syndrome, vasculitis and rheumatological disease due to the deposition of crystalglobulins in various organs. Our case demonstrates that timely investigation with kidney biopsy and appropriate treatment can lead to remission of multiple myeloma and excellent recovery of kidney function.