PMID- 25382003 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20181202 IS - 0042-773X (Print) IS - 0042-773X (Linking) VI - 60 IP - 10 DP - 2014 Oct TI - [Bortezomib-based therapy in patients with light chain deposition disease]. PG - 821-6 AB - Light chain deposition disease (LCDD) is a rare systemic condition caused by monoclonal proliferation of terminally differentiated B-lymphocytes with production of free light chains and their deposition in kidneys or other organs. The aim of our study is to show the pitfalls of the diagnostics, and to demonstrate the effect of bortezomib-based therapy on a series of 4 patients with LCDD, from the point of hematological and organ therapeutic response. We include that bortezomib based treatment provides rapid and effective hematological response. It is, however, often accompanied by adverse events, especially within intensive treatment schedules. The most serious adverse effects includes peripheral neuropathy, which might be dose or treatment-limiting. Less intensive regimens ("bortezomib weekly") suggest an alternative with expectation of lower incidence of adverse effects. Autologous stem cell transplantation is a recommended and relatively safe approach in convenient candidates. Organ response is significantly delayed after hematological response, and organ damage by light chain deposits might not be fully reversible. FAU - Minarik, Jiri AU - Minarik J FAU - Tichy, Tomas AU - Tichy T FAU - Pika, Tomas AU - Pika T FAU - Bacovsky, Jaroslav AU - Bacovsky J FAU - Adamova, Dagmar AU - Adamova D FAU - Srovnalik, Karel AU - Srovnalik K FAU - Krejci, Karel AU - Krejci K FAU - Zadrazil, Josef AU - Zadrazil J FAU - Scudla, Vlastimil AU - Scudla V LA - cze PT - Journal Article PT - Research Support, Non-U.S. Gov't TT - Lecba bortezomibem u pacientu s onemocnenim z depozice lehkych retezcu imunoglobulinu. PL - Czech Republic TA - Vnitr Lek JT - Vnitrni lekarstvi JID - 0413602 RN - 0 (Antineoplastic Agents) RN - 0 (Boronic Acids) RN - 0 (Immunoglobulin Light Chains) RN - 0 (Pyrazines) RN - 69G8BD63PP (Bortezomib) SB - IM MH - Adult MH - Antineoplastic Agents/administration & dosage/*therapeutic use MH - Boronic Acids/administration & dosage/*therapeutic use MH - Bortezomib MH - Female MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - *Immunoglobulin Light Chains MH - Male MH - Middle Aged MH - Paraproteinemias/complications/*drug therapy MH - Pyrazines/administration & dosage/*therapeutic use MH - Treatment Outcome EDAT- 2014/11/11 06:00 MHDA- 2015/10/27 06:00 CRDT- 2014/11/11 06:00 PHST- 2014/11/11 06:00 [entrez] PHST- 2014/11/11 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 50177 [pii] PST - ppublish SO - Vnitr Lek. 2014 Oct;60(10):821-6.