Biomaterial Database

Combined bilateral submandibular and sublingual swelling, macroglossus, and carpal tunnel syndrome caused by light chain amyloidosis. 1998

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Department of Hematology, University of Copenhagen, Denmark.

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.

UI	MeSH Term	Description	Entries
D007147	Immunoglobulin Light Chains	Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. There are two major types of light chains, kappa and lambda. Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule.	Ig Light Chains,Immunoglobulins, Light-Chain,Immunoglobulin Light Chain,Immunoglobulin Light-Chain,Light-Chain Immunoglobulins,Chains, Ig Light,Chains, Immunoglobulin Light,Immunoglobulins, Light Chain,Light Chain Immunoglobulins,Light Chain, Immunoglobulin,Light Chains, Ig,Light Chains, Immunoglobulin,Light-Chain, Immunoglobulin
D008297	Male		Males
D008336	Mandibular Diseases	Diseases involving the MANDIBLE.	Disease, Mandibular,Diseases, Mandibular,Mandibular Disease
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009101	Multiple Myeloma	A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.	Myeloma, Plasma-Cell,Kahler Disease,Myeloma, Multiple,Myeloma-Multiple,Myelomatosis,Plasma Cell Myeloma,Cell Myeloma, Plasma,Cell Myelomas, Plasma,Disease, Kahler,Multiple Myelomas,Myeloma Multiple,Myeloma, Plasma Cell,Myeloma-Multiples,Myelomas, Multiple,Myelomas, Plasma Cell,Myelomas, Plasma-Cell,Myelomatoses,Plasma Cell Myelomas,Plasma-Cell Myeloma,Plasma-Cell Myelomas
D011237	Predictive Value of Tests	In screening and diagnostic tests, the probability that a person with a positive test is a true positive (i.e., has the disease), is referred to as the predictive value of a positive test; whereas, the predictive value of a negative test is the probability that the person with a negative test does not have the disease. Predictive value is related to the sensitivity and specificity of the test.	Negative Predictive Value,Positive Predictive Value,Predictive Value Of Test,Predictive Values Of Tests,Negative Predictive Values,Positive Predictive Values,Predictive Value, Negative,Predictive Value, Positive
D002349	Carpal Tunnel Syndrome	Entrapment of the MEDIAN NERVE in the carpal tunnel, which is formed by the flexor retinaculum and the CARPAL BONES. This syndrome may be associated with repetitive occupational trauma (CUMULATIVE TRAUMA DISORDERS); wrist injuries; AMYLOID NEUROPATHIES; rheumatoid arthritis (see ARTHRITIS, RHEUMATOID); ACROMEGALY; PREGNANCY; and other conditions. Symptoms include burning pain and paresthesias involving the ventral surface of the hand and fingers which may radiate proximally. Impairment of sensation in the distribution of the median nerve and thenar muscle atrophy may occur. (Joynt, Clinical Neurology, 1995, Ch51, p45)	Compression Neuropathy, Carpal Tunnel,Entrapment Neuropathy, Carpal Tunnel,Amyotrophy, Thenar, Of Carpal Origin,Median Neuropathy, Carpal Tunnel,Carpal Tunnel Syndromes,Syndrome, Carpal Tunnel,Syndromes, Carpal Tunnel
D004487	Edema	Abnormal fluid accumulation in TISSUES or body cavities. Most cases of edema are present under the SKIN in SUBCUTANEOUS TISSUE.	Dropsy,Hydrops,Anasarca
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Bilateral carpal tunnel syndrome caused by Graves' disease.

February 1985, Archives of internal medicine,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Senile systemic amyloidosis presenting as bilateral carpal tunnel syndrome.

December 2002, Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Amyloidosis and carpal-tunnel syndrome.

June 1970, The New England journal of medicine,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Carpal Tunnel Syndrome and Trigger Wrist Caused by Localized Amyloidosis: A Case Report.

December 2017, The journal of hand surgery Asian-Pacific volume,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

[Carpal tunnel syndrome and ATTR-amyloidosis].

September 2018, Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V...,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Amyloidosis and the carpal tunnel syndrome.

May 1974, American journal of clinical pathology,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Idiopathic carpal tunnel syndrome caused by carpal stenosis.

May 1980, British medical journal,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Carpal tunnel syndrome caused by cysticercosis.

July 2010, Indian journal of plastic surgery : official publication of the Association of Plastic Surgeons of India,

V Jønsson, and N Rasmussen, and B R Juhl, and P Gimsing, and S Vorstrup

Senile systemic amyloidosis starting as bilateral carpal and left ulnar tunnel syndrome.

October 2003, Internal medicine (Tokyo, Japan),