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Cell Injury

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  1. 1. AmyloidosisAmyloidosis Dr. Deepak K. Gupta
  2. 2. Amyloidosis • Extracellular deposition of fibrillar proteinaceous substance called amyloid – a ‘waxy substance’ composed essentially of an abnormal proteinprotein – Particularly around the supporting fibres of blood vessels and basement membranes. • Associated with a number of inherited and inflammatory disorders Primary Amyloidosis of kidneys
  3. 3. Structure of Amyloid Electron micrograph of 7.5-10 nm amyloid fibrils. Congo red staining shows an apple-green birefringence under polarized light, a diagnostic feature of amyloid.
  4. 4. Types of Amyloid • Based on the biochemical analysis, its composed of 2 main types of complex proteins. • Fibril proteins: comprise about 95% of amyloid. – AL (amyloid light chain) protein – AA (amyloid associated) protein – Other proteins (Transthyretin (TTR), Aβ2-microglobulin (Aβ2M), β-amyloid protein (Aβ), Immunoglobulin heavy chain – Other proteins (Transthyretin (TTR), Aβ2-microglobulin (Aβ2M), β-amyloid protein (Aβ), Immunoglobulin heavy chain amyloid (AH)) • Non-fibrillar components: which include P-component predominantly; constitute the remaining 5% of amyloid – Amyloid P (AP)-component – Apolipoprotein-E (apoE) – α-1 anti-chymotrypsin. – Protein X.
  5. 5. PATHOGENESIS OF AMYLOIDOSIS • Result of immunologic mechanisms. • Multifactorial and that different mechanisms are involved in different types of amyloid. • Fundamentally a disorder of protein misfolding. • Fundamentally a disorder of protein misfolding. • More than 20 (at last count, 23) different proteins can aggregate and form fibrils with the appearance of amyloid • The dye Congo red binds to these fibrils and produces a red-green dichroism (birefringence) – commonly used to identify amyloid desposits in
  7. 7. CLASSIFICATION OF AMYLOIDOSIS • Based on cause – Primary : with unknown cause and the deposition is in the disease itself – Secondary: as a complication of some underlying known disease • Based on extent of amyloid deposition – Systemic (generalised) involving multiple organs– Systemic (generalised) involving multiple organs – Localised amyloidosis involving one or two organs or sites • Based on histological basis – Pericollagenous: corresponding in distribution to primary amyloidosis – Perireticulin: corresponding in distribution to secondary amyloidosis
  8. 8. CLASSIFICATION OF AMYLOIDOSIS • Based on clinical location, – Pattern I: involving tongue, heart, bowel, skeletal and smooth muscle, skin and nerves), – Pattern II: principally involving liver, spleen, kidney and adrenals – Mixed pattern : involving sites of both pattern I and II– Mixed pattern : involving sites of both pattern I and II • Based on tissues in which amyloid is deposited, – Mesenchymal : organs derived from mesoderm – Parenchymal: organs derived from ectoderm and endoderm • Based on precursor biochemical proteins, into specific type of serum amyloid proteins.
  9. 9. CLASSIFICATION OF AMYLOIDOSIS (AL= Amyloid light chain; AA= Amyloid-associated protein; Aβ2M= Amyloid β2- microglobulin; ATTR= Amyloid transthyretin; APrP=Amyloid of prion proteins, Aβ= β- amyloid protein).
  10. 10. STAINING CHARACTERISTICS OF AMYLOID • STAIN ON GROSS – Virchow – frozen/paraffin section – Lugol’s iodine imparts mahogany brown colour to the amyloidthe amyloid – on addition of dilute sulfuric acid turns blue. • H & E – extracellular, homogeneous, structureless – Eosinophilic hyaline material, especially in relation to blood vessels
  11. 11. DIAGNOSIS OF AMYLOIDOSIS • BIOPSY EXAMINATION: commonest and confirmatory method for diagnosis in a suspected case of amyloidosis. • IN VIVO CONGO RED TEST: confirmatory• IN VIVO CONGO RED TEST: confirmatory • OTHER TESTS – supportive of amyloid disease – electrophoresis, immunoelectrophoresis of urine and serum, and bone marrow aspiration
  12. 12. Pathological effects of Amyloidosis AMYLOID DEPOSITION Pressure on adjacent cells Blood vessels Pressure on adjacent cells Blood vessels Atrophy Narrowing Increased permeability Transudation of protein out of vessels
  13. 13. MORPHOLOGIC FEATURES OF AMYLOIDOSIS OF ORGANS • Different organs shows variation in morphologic pattern, some features are applicable in general to most of the involved organs • Most commonly amyloid deposits appear at the contacts between the vascular spaces and parenchymal cells • Grossly• Grossly – affected organ is usually enlarged, pale and rubbery – Cut surface shows firm, waxy and translucent parenchyma – positive staining with the iodine test. • Microscopically – the deposits of amyloid are found in the extracellular locations, initially in the walls of small blood vessels producing microscopic changes and effects, – the deposits are in large amounts causing macroscopic changes and effects of pressure atrophy.
  14. 14. Amyloidosis of kidney The kidney is small and pale in colour. Sectioned surfaceSectioned surface shows loss of cortico- medullary distinction (arrow) and pale, waxy translucency.
  15. 15. Amyloidosis of kidney The amyloid deposits are seen mainly in the glomerular capillary tuft. The deposits are also present in peritubular connective tissue producing atrophic tubules and amyloid casts in the tubular lumina, and in the arterial wall producing luminal narrowing.
  16. 16. Amyloidosis of the spleen
  17. 17. Lardaceous amyloidosis of the spleen The sectioned surface shows presence of plae waxy translucency in a map-liketranslucency in a map-like pattern
  18. 18. Amyloidosis spleen A, The pink acellular amyloid material is seen in the red pulp causing atrophy of while pulp. B, Congo red staining shows Congophilia as seen by red-pink colour. C, When viewed under polarising microscopy the corresponding area shows apple-green
  19. 19. Amyloidosis of the liver A, The deposition is extensive in the space of Disse causing compression and pressure atrophy of hepatocytes. B, Congo red staining shows congophilia which under polarising microscopy. C, shows apple-green
  20. 20. References • Robbinson's basic pathology 8 ed • Harsh Mohan - Textbook of Pathology 6th Ed. • Color atlas of pathology
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