scholarly journals Long-term reversibility of renal dysfunction associated to light chain deposition disease with bortezomib and dexamethasone and high dose therapy and autologous stem cell transplantation

2011 ◽  
Vol 1 (4) ◽  
Author(s):  
Tomás J. González-López ◽  
Lourdes Vázquez ◽  
Teresa Flores ◽  
Jesus Fernando San Miguel ◽  
Ramon García-Sanz
Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5518-5518
Author(s):  
Elizabeth C. Lorenz ◽  
Morie A. Gertz ◽  
Fernando C. Fervenza ◽  
Nelson Leung

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


2019 ◽  
Vol 55 (5) ◽  
pp. 891-905 ◽  
Author(s):  
Hanne Skjerven Bersvendsen ◽  
Hege Sagstuen Haugnes ◽  
Alv A. Dahl ◽  
Unn-Merete Fagerli ◽  
Øystein Fluge ◽  
...  

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