PMID- 1621676
OWN - NLM
STAT- MEDLINE
DCOM- 19920806
LR  - 20190815
IS  - 0272-6386 (Print)
IS  - 0272-6386 (Linking)
VI  - 20
IP  - 1
DP  - 1992 Jul
TI  - Long-term follow-up and response to chemotherapy in patients with light-chain 
      deposition disease.
PG  - 34-41
AB  - Nineteen patients with light-chain deposition disease (LCDD) were studied 
      retrospectively. This report presents data on long-term patient and renal 
      survival and the response to intermittent administration of melphalan and 
      prednisone. Immunoelectrophoresis or immunofixation demonstrated a monoclonal 
      protein in the serum of 78% and in the urine of 84% of the patients; 16% had no 
      demonstrable monoclonal protein in serum or urine. The median age at presentation 
      was 51 years (range, 37 to 77 years). Twelve (63%) of the patients had a 
      monoclonal protein of undetermined significance without evidence of myeloma. The 
      typical glomerular lesion was a diffuse mesangial nodular lesion that was 
      positive for periodic acid-Schiff (PAS) stain with acute and chronic 
      tubulointerstitial changes. Fifteen patients had kappa light-chain deposition and 
      four had lambda light-chain deposition. Five-year actuarial patient survival and 
      survival free of end-stage renal disease were 70% and 37%, respectively. 
      Seventeen patients received melphalan and prednisone, and one patient received 
      chlorambucil and prednisone. All of the patients had some impairment of renal 
      function at presentation, and 58% had a serum creatinine concentration greater 
      than 354 mumol/L (4.0 mg/dL). There was either stabilization or improvement in 
      renal function after chemotherapy in five of eight patients who had a serum 
      creatinine concentration less than 354 mumol/L (4.0 mg/dL) at the initiation of 
      therapy. Of the 11 patients with a high serum creatinine concentration (greater 
      than 354 mumol/dL [4.0 mg/dL]), 82% progressed to end-stage renal disease despite 
      therapy. Follow-up urine protein studies demonstrated at least a 50% decrease in 
      urine protein excretion in five of 15 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
FAU - Heilman, R L
AU  - Heilman RL
AD  - Section of Nephrology and Hypertension, Mayo Clinic Scottsdale, AZ 85259.
FAU - Velosa, J A
AU  - Velosa JA
FAU - Holley, K E
AU  - Holley KE
FAU - Offord, K P
AU  - Offord KP
FAU - Kyle, R A
AU  - Kyle RA
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Am J Kidney Dis
JT  - American journal of kidney diseases : the official journal of the National Kidney 
      Foundation
JID - 8110075
RN  - 0 (Immunoglobulin Light Chains)
RN  - Q41OR9510P (Melphalan)
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Drug Therapy, Combination
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Hypergammaglobulinemia/*drug therapy/mortality
MH  - Immunoglobulin Light Chains/*immunology
MH  - Kidney Diseases/*drug therapy/immunology/mortality
MH  - Male
MH  - Melphalan/*therapeutic use
MH  - Middle Aged
MH  - Prednisone/*therapeutic use
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Time Factors
EDAT- 1992/07/01 00:00
MHDA- 1992/07/01 00:01
CRDT- 1992/07/01 00:00
PHST- 1992/07/01 00:00 [pubmed]
PHST- 1992/07/01 00:01 [medline]
PHST- 1992/07/01 00:00 [entrez]
AID - S0272638692000842 [pii]
AID - 10.1016/s0272-6386(12)80314-3 [doi]
PST - ppublish
SO  - Am J Kidney Dis. 1992 Jul;20(1):34-41. doi: 10.1016/s0272-6386(12)80314-3.