AL amyloidosis

Description

AL (Amyloid Light-Chain) Amyloidosis is due to the deposition of immunoglobulin light chains in glomeruli where they are seen as Congo red binding fibrils and immuno-stain specifically for kappa or lambda light chains. By light microscopy there is amorphous hyaline material in the mesangium and capillary walls. A light chain producing plasma cell or B-cell dysplasia is responsible. Other organs are also involved in this systemic disease.

Index terms

AL amyloidosis
AL [Amyloid Light-Chain]
Immunoglobulinic amyloidosis

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Primary amyloidosis
Organ-limited amyloidosis
AD type amyloidosis
localised amyloid deposit
localised amyloid infiltration
localised amyloidosis
localised generalised amyloid infiltration
localised generalised amyloidosis
localised primary amyloidosis
Cardiac organ-limited amyloidosis
Stiff heart syndrome
amyloid cardiomyopathy
amyloid heart
amyloid heart degeneration
amyloid heart disease
amyloid heart muscle disease
cardiac amyloidosis
familial amyloid cardiomyopathy
familial amyloid heart disease
heart amyloidosis
senile cardiac amyloidosis
Danish type familial amyloid cardiomyopathy
Glomerular disorders in organ-limited amyloidosis
amyloid nephritis
nephrotic syndrome in amyloidosis
Amyloidosis of liver
hepatic amyloidosis
Amyloidosis of small intestine
Amyloid gastrointestinal disease
amyloid intestinal degeneration
Primary localised cutaneous amyloidosis
Macular amyloidosis
Papular amyloidosis
Lichen amyloidosis
Nodular amyloidosis
Amyloidosis cutis nodularis atrophicans
Tumefactive amyloidosis
Interstitial lung diseases associated with amyloidosis
pulmonary amyloidosis
Hepatic amyloidosis with intrahepatic cholestasis
Cholestatic hepatic amyloidosis
Arthropathy in amyloidosis
amyloid arthritis
amyloid arthropathy
arthritis secondary to amyloidosis
articular amyloid
Glaucoma in amyloidosis
Amyloid spinal degeneration
Amyloid splenic degeneration
amyloid splenic disease
AS transthyretin amyloidosis
Danish type amyloidosis
Denmark type amyloidosis
ALECT2 amyloidosis

Exclusions from above levels

6D80 – Dementia due to Alzheimer disease
2B31.2 – histiocytosis X (chronic)
3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency

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5A71.01 – Congenital adrenal hyperplasia
5A81.1 – 5-alpha-reductase deficiency
LD28.01 – Marfan syndrome
LD28.1 – Ehlers-Danlos syndrome
LD2A.4 – androgen resistance syndrome
18 – Pregnancy, childbirth or the puerperium
http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Foundation children elsewhere

8B22.3 – Isolated cerebral amyloid angiopathy

Sibling codes

5D00.1 – AA amyloidosis
5D00.2 – Hereditary amyloidosis
5D00.3 – Dialysis-associated amyloidosis
5D00.Y – Other specified amyloidosis
5D00.Z – Amyloidosis, unspecified

Foundation entity

AL amyloidosis – http://id.who.int/icd/entity/1061366491

Allowed postcoordination axes

Part of: 5D00: Amyloidosis › 05: Endocrine, nutritional or metabolic diseases