Amyloidosis of the tongue as a paraneoplastic marker of plasma cell dyscrasia.
OBJECTIVE
Our objective was to study the results of the medical workup in patients with amyloidosis of the oral cavity.


METHODS
Patients diagnosed with amyloidosis of the oral cavity during the period from January 1971 to January 2001 at the Departments of Oral and Maxillofacial Surgery/Oral Pathology and Dermatology of the VU University Medical Center, Amsterdam, The Netherlands, were included in this retrospective case study. In total, this series comprised 11 patients, 9 women and 2 men. The patients' medical workup and final diagnoses were traced by means of the medical records.


RESULTS
All but one patient presented with amyloidosis of the tongue, most of them manifesting as macroglossia. In 7 of the 11 included patients a diagnosis of myeloma could be established shortly after their referral to the above-stated departments. Three of the 4 remaining patients appeared to have a monoclonal gammopathy of undetermined significance, and 1 patient was diagnosed with a lymphoplasmacytoid non-Hodgkin lymphoma (immunocytoma).


CONCLUSIONS
Amyloidosis of the oral cavity predominantly involves the tongue, mainly manifesting as macroglossia. Amyloidosis of the tongue is associated with an occult underlying plasma cell dyscrasia, in particular myeloma, and, therefore, should be regarded as a paraneoplastic phenomenon of these hematologic diseases.
van der Waal
Rutger I F
RI
Department of Dermatology, Vu University Medical Center/ACTA, Amsterdam, The Netherlands. i.vanderwaal@vumc.nl
van de Scheur
Martijn R
MR
Huijgens
Peter C
PC
Starink
Theo M
TM
van der Waal
Isaäc
I
D000368:Aged; D000369:Aged, 80 and over; D000682:Amyloid / Q000145:classification; D000686:Amyloidosis / Q000175:diagnosis*; D001402:B-Lymphocytes / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis; D008260:Macroglossia / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis; D009101:Multiple Myeloma / Q000175:diagnosis; D010257:Paraneoplastic Syndromes / Q000175:diagnosis*; D010265:Paraproteinemias / Q000175:diagnosis*; D012189:Retrospective Studies; D014060:Tongue Diseases / Q000175:diagnosis*
D000682:Amyloid; D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains