Combined bilateral submandibular and sublingual swelling, macroglossus, and carpal tunnel syndrome caused by light chain amyloidosis.
Three cases of light chain kappa amyloidosis in multiple myeloma patients are described with remarkable involvement of the tongue and swelling of the sublingual and submandibular regions, and without signs of nephropathy despite Bence Jones kappa proteinuria. All three patients had carpal tunnel syndrome at the beginning of their disease course and only moderate gastrointestinal involvement. Primarily for prognostic reasons, amyloidosis should be suspected in such cases, even in the presence of these highly unusual manifestations, and the diagnosis should be confirmed by unambigously-positive biopsies.
Jønsson
V
V
Department of Hematology, University of Copenhagen, Denmark.
Rasmussen
N
N
Juhl
B R
BR
Gimsing
P
P
Vorstrup
S
S
D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis*; D001706:Biopsy; D002349:Carpal Tunnel Syndrome / Q000150:complications* / Q000175:diagnosis; D004487:Edema / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D008297:Male; D008336:Mandibular Diseases / Q000150:complications* / Q000175:diagnosis; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis; D011237:Predictive Value of Tests; D014060:Tongue Diseases / Q000150:complications* / Q000175:diagnosis
D007147:Immunoglobulin Light Chains