Abstract: SA-PO241
Type 1 Cryoglobulinemia with Skin, Nerve, Pulmonary, and Renal Involvement Preceding a Diagnosis of Multiple Myeloma
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Narra, Akshita, Oregon Health and Science University , Portland, Oregon, United States
- Andeen, Nicole K., Oregon Health and Science University , Portland, Oregon, United States
- Avasare, Rupali S., Oregon Health and Science University , Portland, Oregon, United States
Introduction
Type 1 cryoglobulinemia is a rare cause of glomerulonephritis(GN). Here we present a case of a patient with type 1 cryoglobulinemic vasculitis with multiorgan involvement who met criteria for multiple myeloma 1.5 years after initial presentation.
Case Description
A 56-year-old Caucasian male presented with progressively worsening bilateral lower extremity pain and numbness followed by a lacey purple rash and small volume hemoptysis. Serologic workup was negative for ANA, anti-cardiolipin-ab, ANCA, anti-GBM ab, hepatitis B/C, RF, CCP, and cryoglobulin. He had low C3(73mg/dL),normal C4,elevated M protein(0.3gm/dL),elevated serum kappa(175mg/dL) and normal lambda light chains.Bone marrow (BM) biopsy showed 5% monoclonal plasma cells. BM and fat pad biopsies were negative for amyloid. Bronchoscopy was unrevealing.Skin biopsy was inconclusive, but was notable for leucocytoclastic vasculitis vs thrombotic process.He was initiated on high dose prednisone and rituximab therapy.His symptoms waxed and waned and ultimately he developed hematuria, nephrotic-range proteinuria, and acute kidney injury (creatinine 2.3mg/dL) a year later.Kidney biopsy revealed segmental endocapillary hypercellularity, sub endothelial and mesangial eosinophilic immune deposits with 20% crescents.IF was positive for IgG(3+), kappa light chain(2-3+), and C3(1+).EM showed organized immune deposits with straight to slightly curved rods 10-17 nm in diameter very characteristic of cryoglobulinemic GN.A repeat BM biopsy showed expansion of the monoclonal plasma clone to 20%.He was initiated on plasmapheresis, steroids, cyclophosphamide, and carfilzomib with improvement in his symptoms and renal function. Repeat serum cryoglobulin and cryofibrinogen remained undetectable.
Discussion
This case highlights the challenges with diagnosing cryoglobulinemia based on serologic tests alone, and the differential diagnosis and treatment of cryoglobulinemia due to multiple myeloma.We emphasize therapy should be directed toward the causative plasma cell clone, which in this case was rituximab resistant. We present good outcomes after an induction therapy with steroids and plasmapheresis for a rapidly progressive GN due to cryoglobulinemia. Finally, we present a regimen of steroids, cyclophosphamide and carfilzomib in place of bortezomib for patients with significant nerve involvement.