Marked improvement by high-dose chemotherapy and autologous stem cell transplantation in a case of light chain deposition disease

2011 ◽  
Vol 24 (2) ◽  
pp. 246-249 ◽  
Author(s):  
Keiichi Matsuzaki ◽  
Isao Ohsawa ◽  
Tomohito Nishitani ◽  
Yukihiko Takeda ◽  
Hiroyuki Inoshita ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Autologous Stem Cell Transplantation ◽  
Light Chain ◽  
Marked Improvement ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Light Chain Deposition Disease ◽  
Light Chain Deposition

Long-Term Renal Outcome of Autologous Stem Cell Transplantation in Light Chain Deposition Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5518-5518
Author(s):  
Elizabeth C. Lorenz ◽  
Morie A. Gertz ◽  
Fernando C. Fervenza ◽  
Nelson Leung
Keyword(s):  
Renal Function ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Autologous Stem Cell Transplantation ◽  
Prediction Equation ◽  
Light Chains ◽  
High Dose ◽  
Light Chain Deposition Disease ◽  
Light Chain Deposition

Abstract Light chain deposition disease (LCDD) is a systemic disorder characterized by deposition of nonamyloid monotypic light chains throughout the body. Renal dysfunction is a ubiquitous manifestation of the disease. Reports suggest that high-dose chemotherapy and autologous stem cell transplantation may be beneficial in the treatment of LCDD. However, the impact of autologous stem cell transplantation on renal function is unclear. This study retrospectively reviewed the effect of autologous stem cell transplantation on renal function in six patients with LCDD. Two patients had concurrent multiple myeloma, and one patient was on hemodialysis prior to transplantation. Patients received chemotherapy with dexamethasone alone or dexamethasone plus thalidomide prior to transplantation. Conditioning was performed with high-dose intravenous melphalan. All patients survived transplantation, but one sustained multi-organ failure shortly afterwards and died. Another patient required temporary hemodialysis following transplantation. All surviving patients achieved a hematologic response, although one ultimately relapsed and required further chemotherapy. Renal parameters also improved. On average, proteinuria levels decreased by 83% in four patients and increased by 1% in one patient. Creatine clearance calculated by the Modification of Diet in Renal Disease (MDRD) Study prediction equation improved by an average of 40%. This review suggests that autologous stem cell transplantation may be an effective therapy for renal dysfunction associated with LCDD. Clinical Characteristics of LCDD Patients at Presentation Characteristic Number or range Median *Modification of Diet in Renal Disease Study prediction equation Sex 4 male, 2 female Age (years) 35-61 44 Beta-2 microglobulin, μ/ml 3.58-11 4.12 Albumin, g/dl 1.8-4.2 3.85 Creatinine, mg/dl 1.8-5.4 2.45 MDRD*, ml/min 12-50 26.5 Serum M protein, g/dl 0 0 Serum free light chains, mg/dl (n = 5) 2.18-312 19.6 24-hour urine protein, mg 131-10,251 679

Longterm Outcomes and Improved Renal Function with Autologous Stem Cell Transplantation (ASCT) in Light Chain Deposition Disease (LCDD)

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1198-1198
Author(s):  
Kate Stringaris ◽  
Rabya Sayed ◽  
Helen J Lachmann ◽  
Julian D Gillmore ◽  
Philip N Hawkins ◽  
...  
Keyword(s):  
Renal Function ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Autologous Stem Cell Transplantation ◽  
Light Chain ◽  
Organ Function ◽  
Light Chain Deposition Disease ◽  
Light Chain Deposition ◽  
Related Mortality

Abstract Background: Light chain deposition disease (LCDD) is a systemic disorder characterized by monoclonal light chain deposition in organs, most often the kidney but also in the liver, heart and nervous system. Treatment aims to suppress the monoclonal plasma cell population producing the light chains to allow for improvement in or delay deterioration of organ function. Treatments are generally modeled on those for myeloma and light chain amyloidosis. The options are cyclical chemotherapy lately using a novel agent based conditioning regime and, in selected patients, high dose melphalan followed by autologous stem cell transplantation. Due to LCDD being an immunoglobulin disease, based on experience with systemic AL amyloidosis, there is a reluctance to consider ASCT due perceived risks of morbidity and transplant related mortality. There is limited data on the safety and outcomes after ASCT in LCDD. We report here the role of autologous stem cell transplantation to treat LCDD in the largest cohort of patients to date. Methods: This study included all patients between 2003 and 2013 with LCDD who had undergone ASCT from the database of the UK National Amyloidosis Centre (NAC). Data on disease status (clonal markers and organ involvement) at diagnosis, pre and post ASCT was collected from the respective transplant centres for patient progress during and immediately after ASCT. Organ function and clonal response data were serially collected for all patients including clinical course, renal, cardiac and neurological function. Results: A total of ten patients with LCDD were identified who underwent ASCT. This accounted for 23% of all LCDD patients seen at the NAC over this time period. The baseline characteristics were: median age 48 (range 36-60), eGFR 17 ml/min (<10-51), Alkaline phosphatise 92 (51-334), LV wall thickness 10 mm, NT-proBNP 1121 (range 318-4346). Serum monoclonal M protein was quantifiable in 2/10 patients. 9/10 patients had elevated kappa serum free light chains (SFLC) with median level of 1069 mg/dl (range 281-2962) with median κ/λ ratio of 21 (range 0.1-151, NR 0.26-1.65). Only 1/10 had elevated lambda LC. All patients had clonal plasma cells on marrow aspirate with 5/10 patients having >10% (median 15; range 12-50%) and 5/10 , </= 10% (range 2-10%). All patients received chemotherapy prior to ASCT. The median time from diagnosis to ASCT was 13.5 months (Range 4-27). Median follow up post ASCT was 4.8 years (range 6 months-11 years). Haematological responses were seen in all patients with 7/10 acheiving a complete clonal response, 1 patient achieving a VGPR and 2 patients achieving PR. All patients had renal impairment prior to ASCT and 5/10 were on renal dialysis. There was no transplant related mortality and all patients are still alive. After ASCT, 4 patients’ renal function improved, 4/5 remained unchanged on dialysis and 2 patients showed a deterioration in renal function. Of the patients whose renal function improved, one came off dialysis post ASCT, the other three were not on dialysis at any time point. Of the two patients whose renal function deteriorated post ASCT, one required dialysis 3 years after ASCT. Conclusion: This largest study of autologous stem cell transplantation in LCDD to date shows a very high clonal response rate post ASCT. 50% patients had improvement in renal function after transplant. ASCT in LCDD is safe with no transplant related mortality. None of the patients progressed to active myeloma after ASCT. This data supports the use of ASCT in patients with LCDD. Given the rarity of the condition, international registry data would be important to confirm these encouraging findings. Disclosures No relevant conflicts of interest to declare.

scholarly journals Autologous Stem Cell Transplantation in the Treatment of Pulmonary Light Chain Deposition Disease

CHEST Journal ◽  
2021 ◽  
Vol 160 (1) ◽  
pp. e13-e17
Author(s):  
Matthew Rendo ◽  
Teri J. Franks ◽  
Jeffrey R. Galvin ◽  
Andrew Berglund ◽  
Charles Volk ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Autologous Stem Cell Transplantation ◽  
Light Chain ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition
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