[The concurrence of light-chain deposition disease, AL-amyloidosis, and cast nephropathy in a patient with multiple myeloma].
Despite of the fact that their clinical manifestations are similar, AL-amyloidosis (AL-A) and light chain deposition disease (LCDD) are individual nosological entities in view of considerable differences in their pathogenesis and pathomorphology. The paper describes a rare case of the concurrence of LCDD and AL-A in a patient with multiple myeloma. Clinically, there was dialysis-dependent renal failure, flail leg syndrome, myocardiopathy, and rhabdomyolysis. At the disease onset, his nephrobiopsy specimen could diagnose LCDD and myeloma or cast nephropathy. The disease was characterized by an aggressive course. Despite the administration of innovative agents, the patient had a short-term remission and died from disease progression. Autopsy additionally revealed amyloid deposition in the heart and kidney. The development of AL-A in the presence of prior LCDD may reflect the progression of the tumor and the appearance of an additional subclone of plasma cells that produce amyloidogenic light chains. The uncommonness of this case is that renal amyloid was found in the tubular casts and absent in the glomeruli, which may be considered as a special form--tubular AL-amyloidosis.
Hematology Research Center, Ministry of Health of Russia, Moscow, Russia.
I G
IG
Rekhtina
S.P. Botkin City Clinical Hospital, Moscow Healthcare Department, Moscow, Russia.
E V
EV
Zakharova
Department of Nephrology, Faculty of Postgraduate Education, A.I. Evdokimov Moscow State University of Medicine and Dentistry, Ministry of Health of Russia, Moscow, Russia.
E S
ES
Stolyarevich
Hematology Research Center, Ministry of Health of Russia, Moscow, Russia.
M N
MN
Sinitsina
Hematology Research Center, Ministry of Health of Russia, Moscow, Russia.
E N
EN
Denisova
D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D000075363:Immunoglobulin Light-chain Amyloidosis; D007674:Kidney Diseases / Q000150:complications* / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism
D007147:Immunoglobulin Light Chains