Presentation
RESULTS: Six cases of localized tongue amyloidosis presented to the Mayo Clinic between 1969 and 2011. Mean patient age was 69 years (range, 43-90). Patients presented with asymptomatic tongue mass(es). [ncbi.nlm.nih.gov]
Non specific presentation delays 1-2 years in reaching the diagnosis. In our case, the presenting symptoms are fatigue, weight loss, breathlessness, reduced taste perception and mucocutaneous presentations. [edoj.org.eg]
A case of localized deposition of systemic amyloidosis secondary to multiple myeloma is reported in a 63-year-old female presenting with restricted tongue movement in the dental setting. [dental-update.co.uk]
The other two patients without myeloma presented with typical waxy lesions on the face. [8] Our case also presented with typical purpuric patches and waxy nodules around the eyes, nose, and mouth. [jdrntruhs.org]
Entire Body System
- Dentist
Accuracy of entries is verified by an expert review board including dentists and dental hygienists. Colored thumb bleeds make it easy to locate definitions quickly. [books.google.com]
A 66-year-old woman was prompted by her dentist to seek medical attention for an unusually enlarged, smooth-appearing tongue ( Figure 1 ). She also complained of fatigue, dyspnea on exertion, and tingling of her hands. [mdedge.com]
Nonetheless, he had not seen a dentist for this problem or performed any personal oral hygiene. [jmedicalcasereports.biomedcentral.com]
The deposits may be systemic (ie distributed throughout many organs of the body) or localised (ie restricted to single organs such as the lungs, brain, or skin). 6 Most noticeably for the dentist, amyloidosis can affect the mucous membranes of the oral [nature.com]
- Hodgkin Lymphoma
الصفحة 173 - The Non-Hodgkin's Lymphoma Pathologic Classification Project. National Cancer Institute sponsored study of classifications of Non-Hodgkin's lymphomas. Summary and description of a Working Formulation for clinical usage. [books.google.com]
- Lymphadenopathy
The patient presented cervical lymphadenopathy. The incisional biopsy was taken from the tongue and the buccal mucosa. [contempclindent.org]
Liver, Gall & Pancreas
- Hepatomegaly
Hepatomegaly occurs in 50% of patients, Congestive cardiac failure, and nephrotic syndrome in 30% of patients, and splenomegaly occurs in about 10%. Renal involvement manifests as proteinuria and consequently hypoalbuminemia and edema. [jdrntruhs.org]
Gross findings depend on organ involved but may include: Heart - often no gross abnormality; disfunction due to disruption of cardiomyocytes and conduction disturbance Kidney - may have enlarged kidney and/or visible pale deposits in cortex Liver - hepatomegaly [medicine.uiowa.edu]
He had no cardiomegaly, hepatomegaly, splenomegaly or renal involvement. His skiagram chest showed minimal left sided pleural effusion which had no pus cells despite high protein content. [edoj.org.eg]
An enlarged liver (hepatomegaly) and an enlarged spleen (splenomegaly) are the most notable signs. [rarediseases.org]
Jaw & Teeth
- Macroglossia
Discussion: While macroglossia is thought to be pathognomonic of AL amyloidosis, we report a case of macroglossia with fibrillar ATTR amyloid deposits diagnosed by immuno-electron microscopy. [bloodjournal.org]
Dent Update 2016; 43: 641-647 Read article Oral medicine: Amyloidosis Presenting as Macroglossia and Restricted Tongue Movement CPD : 0:24 (closed) Self assess Feedback : 0 comments, 0 ratings Abstract: Amyloidosis (AL) is a well recognized cause of macroglossia [dental-update.co.uk]
Macroglossia means an enlarged tongue. It may be absolute (greater than the 95 th centile) or relative (enlarged compared with oral cavity). [radiopaedia.org]
Of course, amyloidosis should not be the first consideration in a case of macroglossia. [the-medical-dictionary.com]
Pale skin, sparse hair, periorbital puffiness, and macroglossia may sometimes progress to a stuporous state and generalized edema. [aafp.org]
- Lesion of the Tongue
Incisional biopsy was done from one of the nodular lesions in the tongue with the diagnostic possibilities of carcinoma tongue/myeloma infiltration. [jomfp.in]
The biopsy of the excised lesion of the tongue revealed subepithelial accumulation of an amorphous, nodular, fibrillar material positive for Congo red. [link.springer.com]
Purpuric lesions were the only presenting symptom of the patient with myeloma, and only on investigations multiple myeloma was detected. [jdrntruhs.org]
The diagnosis was established on the basis of classical cutaneous lesions. Diagnosis was confirmed by tongue biopsy using Haematoxylin and Eosin staining and crystal violet staining. Polarized microscopy was not done because of unavailability. [edoj.org.eg]
- Cheilitis
Fissured tongue may be part of Melkersson-Rosenthal syndrome, the other features being granulomatous cheilitis and transient facial nerve paralysis; this triad is not necessarily present at the same time. [pocketdentistry.com]
Skin
- Papule
Tongue is diffusely enlarged, firm and fissured with hemorrhagic papules, plaques, nodules and bullae. [jdrntruhs.org]
Based upon our patient's history, these papules developed spontaneously or after minor trauma. The enlarged tongue has interfered with speech and swallowing, and caused sleep apnea. [jmedicalcasereports.biomedcentral.com]
[…] enlarged spleen or problems with spleen function (hyposplenism) enlarged liver or liver failure Position of the spleen shown in green Position of the liver shown in red Skin disease: easy bruising lesions on face, upper trunk and sometimes limbs-lumps, papules [amyloidosis.org.uk]
Hemorrhagic papules involving central face and eyelids in amyloidosis For patients with hereditary systemic amyloidosis in whom the amyloidogenic precursor protein is produced solely by the liver, treatment has been revolutionized by liver transplantation [intelligentdental.com]
Waxy-looking raised bumps (papules) may appear on the face and neck, in the groin, armpits, or anal area, and on the tongue or in the ear canals. Swelling, hemorrhage beneath the skin (purpura), hair loss, and dry mouth may also occur. [encyclopedia.com]
- Blister
Fluid retention causing swelling of the legs and edema Shortness of breath Light-headedness or dizziness Hoarseness of voice Tongue may be enlarged and affected by plaques, blood spots or blisters Related Stories Understanding SARS-CoV-2-induced systemic [news-medical.net]
(D) Individual blisters on upper arm The presentation of amyloidosis is broad and depends on the site of amyloid accumulation. The kidney and heart are the most common organs involved. [en.wikipedia.org]
Neurologic
- Dizziness
Disruption of the nervous system Dizziness or nearly fainting when standing up too quickly. This can happen if the condition affects the nerves that control blood pressure and a sudden drop in blood pressure occurs when standing up. [cedars-sinai.edu]
It can slow blood flow to the brain and cause: Confusion Dizziness Symptoms of a stroke, like weakness on one side of the body and slurred speech Patients with these symptoms should call their doctor. [cancer.org]
Fluid retention causing swelling of the legs and edema Shortness of breath Light-headedness or dizziness Hoarseness of voice Tongue may be enlarged and affected by plaques, blood spots or blisters Related Stories Understanding SARS-CoV-2-induced systemic [news-medical.net]
Some of the symptoms may include: ankle and leg swelling (oedema) muscular weakness unexplained weight loss pins and needles or numbness in the extremities memory loss diarrhoea swollen tongue dizziness when standing up suddenly (orthostatic hypotension [betterhealth.vic.gov.au]
If the condition affects nerves that control blood pressure, you may experience dizziness or near fainting when standing too quickly. Amyloidosis care at Mayo Clinic July 07, 2017 References Gorevic PD. [mayoclinic.org]
- Vertigo
Medically the patient suffered occasional migraine-type headaches and had an episode of vertigo six months previously. She had undergone a hysterectomy 22 years previously, was a non-smoker, occasionally drank alcohol but was otherwise fit and well. [nature.com]
Workup
An extensive workup to differentiate systemic and localized amyloidosis required to treat underlying pathology and to prevent organ damage. [saudijos.org]
Serum
- Howell-Jolly Bodies
Splenic dysfunction, leading to the presence of Howell-Jolly bodies on blood smear, occurs in 24% of people with amyloidosis. [6] Malabsorption is seen in 8.5% of AL amyloidosis and 2.4% of AA amyloidosis. [en.wikipedia.org]
Colonoscopy
- Polyps
], [7], [8], [9], [10] Fibroepithelial polyp, lymphangiomas, hemangiomas, and lipomas can also cause similar nodules in oral mucosa. [jomfp.in]
Treatment
Comprehensive amyloidosis treatment Team-based care is essential if amyloidosis affects tissues and organs throughout your body, as it often does. [seattlecca.org]
What is the treatment for amyloidosis? There is no cure for amyloidosis. The treatment of amyloidosis depends on the type of amyloidosis involved. [medicinenet.com]
Treatment for amyloidosis The aims of treatment are to prevent further deposits of amyloid proteins and ease the symptoms. Treatment options depend on the form of amyloidosis, what organs are affected and the associated symptoms. [betterhealth.vic.gov.au]
The genetic testing is required as the course of treatment is completely different for these two forms of the disease. Treatment of Amyloidosis The treatments for each type of amyloidosis differ. [chipmiller.org]
Liver transplantation has been used most successfully for treatment of FAP. [intelligentdental.com]
Prognosis
Objective: To assist GDPs in the detection of early warning signs of multiple myeloma and amyloidosis to help improve the prognosis of patients with these conditions. [dental-update.co.uk]
The prognosis is uncertain, owing to the rarity of the condition, requiring regular follow-up and monitoring. [ncbi.nlm.nih.gov]
The presence of myeloma yields a poor prognosis for the patient. [intelligentdental.com]
The prognosis depends on the type of amyloidosis and the organ system that is affected. Involvement of the heart is the most dangerous form and can have a bleak prognosis. [merckmanuals.com]
Amyloidosis associated by multiple myeloma usually has a poor prognosis. Most patients with both diseases die within one to two years. Prevention Genetic couseling may be helpful for patients with hereditary amyloidosis and their families. [encyclopedia.com]
Etiology
Etiology The etiology of BMS is unknown. Although a variety of causes have been mentioned in the literature, the most common one being an underlying psychiatric disorder, there is no proven causative etiological factor. [pocketdentistry.com]
Our patient's weight loss and the presence of the submandibular mass initially led us to consider a malignant etiology, but this theory was not supported by imaging and laboratory findings. [the-medical-dictionary.com]
He underwent extensive tests to identify the etiologies associated with systemic amyloidosis. [jmedicalcasereports.biomedcentral.com]
It is a plasma cell dyscrasia of unknown etiology with the production of monoclonal chains. [saudijos.org]
[…] least common form, with few previously reported cases. 8 Notably, central nervous system amyloidomas are a different entity from systemic amyloidosis or from the more common congophilic amyloid angiopathy. 27 They occur in isolation and have no known etiologic [ajnr.org]
Epidemiology
Epidemiology The estimated prevalence is approximately 1 %. Geographic tongue may occur on all ages, even in young children. [pocketdentistry.com]
Systemic complications of acromegaly: epidemiology, pathogenesis, and management. Endocr Rev. 2004;25(1):102–152. 5. Xavier SD, Bussoloti IF, Müller H. Macroglossia secondary to systemic amyloidosis: case report and literature review. [aafp.org]
Epidemiology of multiple myeloma. In Wiernik P H, Canellos G P, Kyle P A, Schiffer C A (Eds) Neoplastic diseases of the blood. Vol 1, 2 nd ed. pp 347–372. [nature.com]
Epidemiology United States Annually, one to five cases of immunoglobulin-related amyloidosis per 100,000 people occur. [emedicine.medscape.com]
[…] concentration of serum amyloid A protein. [7] People with ATTR have better prognosis and may survive for over a decade. [6] Senile systemic amyloidosis was determined to be the primary cause of death for 70% of people over 110 who have been autopsied. [20] [21] Epidemiology [en.wikipedia.org]
Pathophysiology
Amyloidosis: a clinic-pathophysiological synopsis. Semin Cell Develop Biol. 2004;15:39–44. CrossRef Google Scholar 3. Westermark P, Benson MD, Buxbaum JN, et al. A primer of amyloid nomeclature. Amyloid. 2007;14:179–83. [link.springer.com]
"Leukocyte Cell-Derived Chemotaxin-2: It's Role in Pathophysiology and Future in Clinical Medicine". Clinical and Translational Science. 10 (4): 249–259. doi : 10.1111/cts.12469. PMC 5504477. [en.wikipedia.org]
Pathophysiology The most common light chain–type amyloidosis precursor proteins are L chains of the lambda (l) class. [emedicine.medscape.com]
Islet amyloid and type 2 diabetes; from molecular misfolding to islet pathophysiology. Biochim Biophys Acta. 2001;1537:179-203. Sanchorawala V, Wright DG, Seldin DC, et al. [rarediseases.org]
Prevention
Prevention Because no one knows what causes amyloidosis, there is no way to prevent primary amyloidosis. Secondary amyloidosis can be prevented only by preventing or promptly treating the inflammatory disease that can trigger amyloidosis. [drugs.com]
These treatments can give your body time to gradually clear the deposits before they build up again, and can help prevent organ damage. In most cases, the treatment will involve having chemotherapy. [nhs.uk]
Is it possible to prevent amyloidosis? There is no prevention for amyloidosis. However, the secondary forms of amyloidosis can be prevented by treating the underlying diseases that are associated with inflammation. [medicinenet.com]
The aims of treatment are to prevent further deposits of amyloid proteins and ease the symptoms. [betterhealth.vic.gov.au]
NCI comprehensive cancer center We are a comprehensive cancer center, a designation from the National Cancer Institute that reflects our scientific leadership and the depth and breadth of our research to prevent, diagnose and treat cancer. [seattlecca.org]