[Bortezomib-based therapy in patients with light chain deposition disease].
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.
Jiří
J
Minařík
Tomáš
T
Tichý
Tomáš
T
Pika
Jaroslav
J
Bačovský
Dagmar
D
Adamová
Karel
K
Srovnalík
Karel
K
Krejčí
Josef
J
Zadražil
Vlastimil
V
Sčudla
D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage / Q000627:therapeutic use*; D000069286:Bortezomib; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy*; D011719:Pyrazines / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D001897:Boronic Acids; D007147:Immunoglobulin Light Chains; D011719:Pyrazines; D000069286:Bortezomib