PMID- 18178602
OWN - NLM
STAT- MEDLINE
DCOM- 20080731
LR  - 20080521
IS  - 1460-2385 (Electronic)
IS  - 0931-0509 (Linking)
VI  - 23
IP  - 6
DP  - 2008 Jun
TI  - Long-term outcome of autologous stem cell transplantation in light chain 
      deposition disease.
PG  - 2052-7
LID - 10.1093/ndt/gfm918 [doi]
AB  - BACKGROUND: Light chain deposition disease (LCDD) is a systemic disorder 
      characterized by deposition of non-amyloid monoclonal light chains. Renal 
      dysfunction is a ubiquitous manifestation of the LCDD disease. Reports suggest 
      that high-dose chemotherapy and autologous stem cell transplantation (ASCT) may 
      be beneficial in the treatment of LCDD. However, the impact of ASCT on renal 
      function is unclear. This study retrospectively reviewed the effect of ASCT on 
      renal function in patients with LCDD. METHODS: Six patients with LCDD have been 
      transplanted at our institution since 2001. Patients received dexamethasone 
      alone, dexamethasone plus thalidomide or no chemotherapy prior to conditioning. 
      All the patients underwent high-dose melphalan conditioning after stem cell 
      mobilization. RESULTS: Three of the six patients had concurrent multiple myeloma 
      (MM), and one patient was on haemodialysis prior to transplantation. Four 
      patients were male and two were female. The median age was 43.5 years with a 
      median serum creatinine of 2.4 mg/dl and a median estimated glomerular filtration 
      rate (eGFR) of 26.5 ml/min/1.73 m(2). Five patients survived ASCT and one died on 
      Day 26 of transplantation. Median follow-up was 31.7 months (range 31.3-60.7 
      months) after ASCT. Of the surviving patients, all the five achieved a 
      haematological response post-transplantation although two ultimately relapsed and 
      required further chemotherapy. The eGFR of one patient declined with relapse and 
      improved with treatment, while the eGFR of the second patient remained stable 
      throughout relapse and treatment. The patient on haemodialysis prior to 
      transplantation continued to require it afterward, but ultimately received a 
      renal transplant. Median reduction in proteinuria was 92% and median improvement 
      in eGFR was 95%. Of the four evaluable patients all achieved criteria for a renal 
      response after ASCT. CONCLUSIONS: ASCT may be an effective therapy for renal 
      dysfunction associated with LCDD. In cases where kidney dysfunction persists 
      after ASCT, a haematological response may permit successful kidney 
      transplantation with improved graft viability and decreased risk of recurrence.
FAU - Lorenz, Elizabeth C
AU  - Lorenz EC
AD  - Division of Nephrology and Hypertension, Mayo Clinic, 200 1st Street SW, 
      Rochester, MN 55905, USA.
FAU - Gertz, Morie A
AU  - Gertz MA
FAU - Fervenza, Fernando C
AU  - Fervenza FC
FAU - Dispenzieri, Angela
AU  - Dispenzieri A
FAU - Lacy, Martha Q
AU  - Lacy MQ
FAU - Hayman, Suzanne R
AU  - Hayman SR
FAU - Gastineau, Dennis A
AU  - Gastineau DA
FAU - Leung, Nelson
AU  - Leung N
LA  - eng
PT  - Journal Article
DEP - 20080104
PL  - England
TA  - Nephrol Dial Transplant
JT  - Nephrology, dialysis, transplantation : official publication of the European 
      Dialysis and Transplant Association - European Renal Association
JID - 8706402
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Female
MH  - Follow-Up Studies
MH  - Graft Rejection
MH  - Graft Survival
MH  - Hematopoietic Stem Cell Mobilization
MH  - Hematopoietic Stem Cell Transplantation/adverse effects/*methods
MH  - Humans
MH  - Immunoglobulin Light Chains
MH  - Male
MH  - Middle Aged
MH  - Multiple Myeloma/diagnosis/mortality/therapy
MH  - Paraproteinemias/diagnosis/*mortality/*therapy
MH  - Renal Insufficiency/diagnosis/mortality/therapy
MH  - Retrospective Studies
MH  - Risk Assessment
MH  - Severity of Illness Index
MH  - Survival Analysis
MH  - Transplantation Conditioning/methods
MH  - Transplantation, Autologous
MH  - Treatment Outcome
EDAT- 2008/01/08 09:00
MHDA- 2008/08/01 09:00
CRDT- 2008/01/08 09:00
PHST- 2008/01/08 09:00 [pubmed]
PHST- 2008/08/01 09:00 [medline]
PHST- 2008/01/08 09:00 [entrez]
AID - gfm918 [pii]
AID - 10.1093/ndt/gfm918 [doi]
PST - ppublish
SO  - Nephrol Dial Transplant. 2008 Jun;23(6):2052-7. doi: 10.1093/ndt/gfm918. Epub 
      2008 Jan 4.