Contributions
Abstract: PB2195
Type: Publication Only
Background
Light-chain deposition disease is characterized by deposition of nonamyloid monoclonal light chains in multiple organs. It is a rare disease caused by overproduction of kappa chains and very rarely lambda chains by a single clone of plasma cells. LCDD can occur in any organ but kidneys are always involved as a rapidly progressive glomerulonephritis or acute tubulointerstitial nephritis. A mandatory step in LCDD diagnosis is identifying the monoclonal pathological light chain in serum or urine. LCDD should be distinguished from other monoclonal proteins associated diseases like cryoglobulinemia, myeloma cast nephropathy, amyloidosis and Fanconi syndrome.
Aims
Large multicenter series of patients are needed to develop an optimal standardized treatment strategy.
Methods
We present a single center experience with twelve cases of LCDD diagnosed betweed 2015 and 2017. We assessed the patients with the same panel of investigations used in the case of amyloidosis, including serum electrophoresis and immunofixation, free light chain assay, proteinuria, fibroscan, echocardiogram, electromyography and whole body-low dose CT. Renal biopsy, bone marrow aspirate and biopsy, abdominal fat biopsy and salivary gland biopsy were performed on every patient. All diagnosis were confirmed following renal biopsy (electronic microscopy and immunofluorescence) that presented glomeruli with dense linear subendothelial deposits in capillary loops; patchy dense deposits ; mezangiosclerosis areas; multiple endothelial detachments; disorganised tubular basal membrane with spidery, linear dense deposits.In our group of patients all abdominal fat biopsies were Congo red stain negative under polarized light.
Results
Eleven of them had kappa LCDD and one presented lambda LCDD. All the patients had a bone marrow plasmacytic infiltrate of under 10%. None of them had osteolytic bone lesions. Three patients had hepatic involvement (Fibroscan > 8,6 kPa ) and one patient presented restrictive cardiomiopathy.Two patients had Sicca Syndrome. Electromyography showed sensitive neuropathy in one patient, chronic motor-sensitive axonal polyneuropathy in one patient and chronic demyelinating polyneuropathy in one patient.
Renal involvement was present in six patients as stage 3 CKD, two had CKD stage 4 and the other four stage 5 CKD. Four patients required hemodyalisis initiation. Three patients presented with nephrotic syndrome and seven patients had subnephrotic range proteinuria. Three had microscopic haematuria. Nine patients presented with secondary hypertension.
Ten patients received Bortezomib-based therapy and two patients received Melphalan based therapy. Two of them underwent autologous bone marrow transplant without complications and without additional renal function reduction.
After the completion of the treatment, in 2018 all twelve patients are alive with controlled disease. None of them required therapy resumption.
Four patients had CR/VGPR and three of them showed renal function improvement. ASCT was performed even in advanced CKD stages.
Conclusion
LCDD should be considered in patients with MGUS and CKD. Bortezomib based therapy followed by ASCT showed promising results.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Autologous hematopoietic stem cell transplantation, Monoclonal gammopathy, Proteasome inhibitor, Renal impairment
Abstract: PB2195
Type: Publication Only
Background
Light-chain deposition disease is characterized by deposition of nonamyloid monoclonal light chains in multiple organs. It is a rare disease caused by overproduction of kappa chains and very rarely lambda chains by a single clone of plasma cells. LCDD can occur in any organ but kidneys are always involved as a rapidly progressive glomerulonephritis or acute tubulointerstitial nephritis. A mandatory step in LCDD diagnosis is identifying the monoclonal pathological light chain in serum or urine. LCDD should be distinguished from other monoclonal proteins associated diseases like cryoglobulinemia, myeloma cast nephropathy, amyloidosis and Fanconi syndrome.
Aims
Large multicenter series of patients are needed to develop an optimal standardized treatment strategy.
Methods
We present a single center experience with twelve cases of LCDD diagnosed betweed 2015 and 2017. We assessed the patients with the same panel of investigations used in the case of amyloidosis, including serum electrophoresis and immunofixation, free light chain assay, proteinuria, fibroscan, echocardiogram, electromyography and whole body-low dose CT. Renal biopsy, bone marrow aspirate and biopsy, abdominal fat biopsy and salivary gland biopsy were performed on every patient. All diagnosis were confirmed following renal biopsy (electronic microscopy and immunofluorescence) that presented glomeruli with dense linear subendothelial deposits in capillary loops; patchy dense deposits ; mezangiosclerosis areas; multiple endothelial detachments; disorganised tubular basal membrane with spidery, linear dense deposits.In our group of patients all abdominal fat biopsies were Congo red stain negative under polarized light.
Results
Eleven of them had kappa LCDD and one presented lambda LCDD. All the patients had a bone marrow plasmacytic infiltrate of under 10%. None of them had osteolytic bone lesions. Three patients had hepatic involvement (Fibroscan > 8,6 kPa ) and one patient presented restrictive cardiomiopathy.Two patients had Sicca Syndrome. Electromyography showed sensitive neuropathy in one patient, chronic motor-sensitive axonal polyneuropathy in one patient and chronic demyelinating polyneuropathy in one patient.
Renal involvement was present in six patients as stage 3 CKD, two had CKD stage 4 and the other four stage 5 CKD. Four patients required hemodyalisis initiation. Three patients presented with nephrotic syndrome and seven patients had subnephrotic range proteinuria. Three had microscopic haematuria. Nine patients presented with secondary hypertension.
Ten patients received Bortezomib-based therapy and two patients received Melphalan based therapy. Two of them underwent autologous bone marrow transplant without complications and without additional renal function reduction.
After the completion of the treatment, in 2018 all twelve patients are alive with controlled disease. None of them required therapy resumption.
Four patients had CR/VGPR and three of them showed renal function improvement. ASCT was performed even in advanced CKD stages.
Conclusion
LCDD should be considered in patients with MGUS and CKD. Bortezomib based therapy followed by ASCT showed promising results.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Autologous hematopoietic stem cell transplantation, Monoclonal gammopathy, Proteasome inhibitor, Renal impairment