Basic Overview

There are several different types of Amyloidosis and this page summarizes them with their treatments. We have not attempted to list all the symptoms, as they are numerous, and can vary greatly. A good overview summary of Amyloidosis can be found at http://emedicine.medscape.com/article/335414-overview.

Throughout this article we have many references to 'www.emedicine.com' pages as they are a reliable source of information.

The various types of Amyloidosis are Primary Amyloidosis (AL), Secondary Amyloidosis (AA), Familial Amyloidosis (FAP in some cases), Cutaneous Amyloidosis, Cerebral/Central Nervous System Amyloid Diseases, Heavy Light Chain Amyloidosis (AH), Beta-2-Microglobulin Amyloidosis (B2M - Dialysis Related), and Localized Amyloidosis.

* Primary Amyloidosis (AL)

-http://emedicine.medscape.com/article/1093258-overview

- Usually has either Lambda or Kappa Free Light Chains in the blood, with also (usually) Bence Jones Protein in the urine.

- It is usually systemic, and any part of the body may be affected, although the amyloid tends to congregate in certain organs/areas whilst missing others, like the brain.

- Usually caused by a plasma cell disorder.

- Up to 31% of Multiple Myeloma (usually with an IgM spike) patients can develop AL Amyloidosis. (taken from Clin Exp Dermatol. 2002 May;27(3):240-2).

http://emedicine.medscape.com/article/208839-overview

- Free Light Chains are present in 98% of patients, and should be initially tested prior to any treatments (especially chemotherapy). An article on Free Light Chains from the Binding Site.

-Also can be developed by Waldenstrom's Macroglobulinemia patients.

- http://emedicine.medscape.com/article/207097-overview

- Rarely caused by a Non-Hodgkin Lymphoma.

-http://emedicine.medscape.com/article/203399-overview

    - Treatment is highly tailored according to the individual patient's criteria (extent of disease, organs affected, age, fitness). Treatment can be a Stem Cell Transplant (SCT) or Chemotherapy (such as Dexamethasone combined with other drugs, or the less aggressive Melphalan with Prednisolone). Occasionally some patient's may require an organ transplant for a damaged organ, especially when combined with a successful SCT.

More information: http://www.amyloidosis.org.uk/al-amyloidosis/understanding-associated-conditions/


* Secondary Amyloidosis (AA) - Inflammatory

- This amyloidosis appears as a result of an underlying existing disease, such as:

- Rheumatoid Arthritis, including Psoriatic Arthritis and Ankylosing Spondylitis

- Crohn's disease

- Leprosy

- Familial Mediterranean Fever (FMF)

- http://www.emedicine.com/med/topic1410.htm

- Other Periodic Familial Fevers (TRAPS, Muckle-Wells, HID's etc)

- https://www.rareconnect.org/en/community/familial-mediterranean-fever

- http://www.amyloidosis.org.uk/fever-syndromes/inherited-fever-syndromes/

- Unknown causes

- Treating the underlying disease successfully usually stops the AA from forming or progressing further.

- http://emedicine.medscape.com/article/335559-overview

- There are existing effective drug therapies for it, such as Colchicine (for FMF), Chlorambucil (for Rheumatoid Arthritis) and newly developed drugs such as Kiacta (formerly known as Fibrillex).

More information:

- http://www.amyloidosis.org.uk/understanding-aa-amyloidosis/aa-amyloidosis/

* Familial (or hereditary) Amyloidosis

- Familial Amyloid Polyneuropathy (FAP) - main symptom is a Polyneuropathy.

- The most common type of Familial Amyloidosis. Includes variants (different genetic mutations) such as Transthyretin (TTR)

-http://emedicine.medscape.com/article/335301-overview

- Treatment is usually a Liver transplant, or newly discovered drug therapies for those not eligible for transplants

- Familial Amyloid Cardiomyopathy (FAC) - main symptom is Cardiomyopathy.

- Present in up to 4% of the African American population

- Renal associated Familial Amyloidosis, such as Fibrinogen A alpha chain, Lyzosyme, Apolipoprotein A-1, etc.

-http://emedicine.medscape.com/article/246221-overview

- Gelsolin Amyloidosis, also known as Familial Amyloidosis Finnish Type (FAF), due to most cases originating in Finland.

More information: http://www.amyloidosis.org.uk/hereditary-systemic-amyloidosis/

* Cutaneous Amyloidosis

- Lichen - usually in males of Chinese origin. Usually not systemic.

http://emedicine.medscape.com/article/1102672-overview

- Macular - usually not systemic. Hyperpigmented itchy macules in the skin.

http://emedicine.medscape.com/article/1102860-overview

- Nodular - very rare. Limited to the skin. Unlikely to become systemic.

http://emedicine.medscape.com/article/1102770-overview

* Cerebral/Central Nervous System Amyloid Diseases

- Alzheimer's Disease - caused by beta Amyloid plaques

http://emedicine.medscape.com/article/1134817-overview

- Cerebral Amyloid Angiopathy (CAA) also caused by beta Amyloid

- http://emedicine.medscape.com/article/1162720-overview

- Prion Protein (PrP) Diseases, such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, etc

http://emedicine.medscape.com/article/1168941-overview

* Heavy Light Chain Amyloidosis (AH)

- Rarely some patients have immunological heavy chain amyloidosis.

- Included in the article at http://emedicine.medscape.com/article/200758-overview

* Beta-2-Microglobulin Amyloidosis (B2M) - Dialysis Related

- Occasionally occurs as a complication of long-term dialysis.

- Treatment is usually a renal/kidney transplant

- http://emedicine.medscape.com/article/246542-overview


* Localized Amyloidosis

- Deposits of amyloid in a single organ or area (eg. the bladder)

- Difficult to determine if it is local to the area or systemic

- Treatment is specific to the individual patient

More information: http://www.amyloidosis.org.uk/other-types-of-amyloidosis/localised-amyloidosis/