EXTRACELLULAR EXTRACELLULAR ACCUMULATIONSACCUMULATIONS

(mesenchimal degenerations, (mesenchimal degenerations,

stromal-vascular degenerations)stromal-vascular degenerations)

Group of metabolic disturbances Group of metabolic disturbances these develop out of cells, in these develop out of cells, in

extracellular matrixextracellular matrix

Classifications:Classifications:- according to type of disturbed - according to type of disturbed metabolism:metabolism:a) protein;a) protein;b) fat;b) fat;c) carbohydrate.c) carbohydrate.

- according to prevalence:- according to prevalence:a) local;a) local;b) general (widespread).b) general (widespread).

- according to influence of according to influence of genetic factors:genetic factors:a) hereditary (tesaurosmoses);a) hereditary (tesaurosmoses);b) acquired. b) acquired.

Morphogenetic mechanisms:Morphogenetic mechanisms:- infiltration;- infiltration;

- decomposition;- decomposition;- abnormal synthesis;- abnormal synthesis;

- transformation.- transformation.

PROTEIN EXTRACELLULAR PROTEIN EXTRACELLULAR ACCUMULATIONSACCUMULATIONS

The group includes:The group includes:

- mucoid swelling;- mucoid swelling;- fibrinoid changes (fibrinoid - fibrinoid changes (fibrinoid

swelling and fibrinoid necrosis);swelling and fibrinoid necrosis);- hyalinosis;- hyalinosis;

- amyloidosis.- amyloidosis.

Mucoid swelling –Mucoid swelling –

superficial and REVERSIBLE superficial and REVERSIBLE desorganisation of connective desorganisation of connective

tissuetissue

Morphology:Morphology:

- increasing of permeability;- increasing of permeability;- redistribution and accumulation - redistribution and accumulation of acid mucopolysacharids of acid mucopolysacharids (GAG);(GAG);- swelling of collagen fibers - swelling of collagen fibers WITHOUT their destruction.WITHOUT their destruction.

GROSS:GROSS:- there are not any changes- there are not any changes

MICROSCOPY:MICROSCOPY:- in H&E it is invisible;- in H&E it is invisible;

- can be diagnosed with help of - can be diagnosed with help of specific histochemical reactions specific histochemical reactions (eg. toluidin blue,PAS-reaction)(eg. toluidin blue,PAS-reaction)

Mucoid swelling of arterial wall in rheumatic fever (PAS)Mucoid swelling of arterial wall in rheumatic fever (PAS)

Mucoid swelling of heart valve in rheumatic feverMucoid swelling of heart valve in rheumatic fever

Mucoid swelling (electron microscopy): swelling of Mucoid swelling (electron microscopy): swelling of collagen fibers WITHOUT their destructioncollagen fibers WITHOUT their destruction

Results (outcomes):Results (outcomes):- restoration- restoration

- coming to fibrinoid changes- coming to fibrinoid changes

Fibrinoid changes (include Fibrinoid changes (include fibrinoid swelling and fibrinoid fibrinoid swelling and fibrinoid

necrosis as deeper destruction of necrosis as deeper destruction of CT) – pathological process CT) – pathological process characterized by deep and characterized by deep and

irreversible desorganisation of irreversible desorganisation of connective tissueconnective tissue

Morphology:Morphology:

- total destruction of collagenic - total destruction of collagenic fibers;fibers;

- total destruction of interfibrilar - total destruction of interfibrilar spaces and extracellular matrix;spaces and extracellular matrix;

- increasing of permeability;- increasing of permeability;- formation of unstructural, fibrin - formation of unstructural, fibrin

contained masses.contained masses.

Gross:Gross:- there are not any changes/- there are not any changes/

Microscopy:Microscopy:- in H&E usually invisible;- in H&E usually invisible;

- can be recognized with help of - can be recognized with help of specific stain (eg. Van Gaison, specific stain (eg. Van Gaison,

Veigert method)Veigert method)

Fibrinoid changes in connective tissue: focus of Fibrinoid changes in connective tissue: focus of desorganisation stained in blue (Veigert)desorganisation stained in blue (Veigert)

Fibrinoid changes (electron microscopy): Fibrinoid changes (electron microscopy): total destruction of collagen fiberstotal destruction of collagen fibers

Results (outcomes):Results (outcomes):- coming to hyalinosis;- coming to hyalinosis;

- sclerosis- sclerosis

Hyalinosis – irreversible, deep Hyalinosis – irreversible, deep destruction of connective tissue, destruction of connective tissue, characterizing by appearance of characterizing by appearance of unstractural, glass-like (hyalin unstractural, glass-like (hyalin

cartilage-like) masses in cartilage-like) masses in destructive foci.destructive foci.

Preceding processes:Preceding processes:- necrosis;- necrosis;

-fibrinoid changes;-fibrinoid changes;- sclerosis;- sclerosis;

- plasmatic infiltration.- plasmatic infiltration.

There ere two types:There ere two types:- vascular (usually widespread)- vascular (usually widespread)

- hyalinosis of connective tissue - hyalinosis of connective tissue (as rule local)(as rule local)

Vascular type takes small Vascular type takes small arteries and arterioles of kidneys, arteries and arterioles of kidneys,

brain, pancreas, retina of eyes, brain, pancreas, retina of eyes, intestine.intestine.

Can be observed in different Can be observed in different chronic hypertensions, chronic hypertensions,

rheumatic fever, diabetes rheumatic fever, diabetes mellitus, chronic infections, mellitus, chronic infections, autoimmune diseases etc.autoimmune diseases etc.

There are three chemical variants There are three chemical variants of vascular hyalin:of vascular hyalin:

- simple (consists of proteins - simple (consists of proteins only, occurs in hypertensions),only, occurs in hypertensions),

- lipohyalin (consists of proteins - lipohyalin (consists of proteins and lipids. In diabetes mellitus)and lipids. In diabetes mellitus)

- complex (proteins+immune - complex (proteins+immune complexes. Rheumatic fever, complexes. Rheumatic fever,

autoimmune diseases)autoimmune diseases)

Connective tissue local hyalinosisConnective tissue local hyalinosis

can be found incan be found in- scars;- scars;

- base of chronic peptic ulcer;- base of chronic peptic ulcer;- in tonsil in chr. tonsillitis;- in tonsil in chr. tonsillitis;

- in appendix in chr. appendicitis;- in appendix in chr. appendicitis;- in casps of heart valve in defective - in casps of heart valve in defective

valvular heart diseases;valvular heart diseases;- in liver and spleen capsules after - in liver and spleen capsules after

purulent peritonitis etc.purulent peritonitis etc.

Morphology:Morphology:

at visual inspection:at visual inspection:hard, semitransparent (glass-like) hard, semitransparent (glass-like) focus. Blood vessels with narrowed focus. Blood vessels with narrowed lumen and thick, hard, glass-like lumen and thick, hard, glass-like walls (like tubules of glass)walls (like tubules of glass)microscopy:microscopy:- in G&E – homogenous, - in G&E – homogenous, eosinophilic (pink), unstructural eosinophilic (pink), unstructural massmass

Hyalinosis of brain arteriole in arterial Hyalinosis of brain arteriole in arterial hypertensionhypertension

Hyalinosis of spleen arterioleHyalinosis of spleen arteriole

Kidney in arterial hypertensionKidney in arterial hypertension

Results (outcomes):Results (outcomes):- long time presence without any - long time presence without any

changeschanges- mucoidization (rare)- mucoidization (rare)-resolution (most rare)-resolution (most rare)

Amyloidosis – deposition of Amyloidosis – deposition of abnormal, compound protein abnormal, compound protein

(amyloid) in organs and tissues(amyloid) in organs and tissues

will be considered later.will be considered later.

Fatty extracellular accumulationsFatty extracellular accumulations

Two forms:Two forms:- disturbances of cholesterol and - disturbances of cholesterol and

its ethers metabolism;its ethers metabolism;- disturbances of neutral fat - disturbances of neutral fat

metabolismmetabolism

The first form occurs in The first form occurs in atherosclerosisatherosclerosisCharacterized by deposition of Characterized by deposition of cholesterol in large arteries cholesterol in large arteries walls.walls.

Aortic wall in atherosclerosis: deposition of Aortic wall in atherosclerosis: deposition of lipoproteins and cholesterol in intima, stain Sudan IIIlipoproteins and cholesterol in intima, stain Sudan III

Neutral fat metabolism Neutral fat metabolism disturbances:disturbances:

there are two variants of the there are two variants of the disorder:disorder:

1. Decreased containment of fat in 1. Decreased containment of fat in fat depot (cachexia or emaciation)fat depot (cachexia or emaciation)2. Increased containment of fat in 2. Increased containment of fat in

fat depot (obesity) fat depot (obesity)

Cachexia can beCachexia can be- alimentary;- alimentary;- cerebral;- cerebral;- neoplastic (in malignant - neoplastic (in malignant tumours);tumours);- endocrine;- endocrine;- in chronic severe diseases;- in chronic severe diseases;- different vitamins - different vitamins insufficiencies;insufficiencies;- chronic intoxications.- chronic intoxications.

Obesity Obesity

Classifications of obesity:Classifications of obesity:- acc. to topography:- acc. to topography:upper, middle, low types; symmetrical, upper, middle, low types; symmetrical, asymmetrical.asymmetrical.- acc. to morphology:- acc. to morphology:hypertrophic, hyperplastic.hypertrophic, hyperplastic.- clinical:- clinical:I, II, III, IV degrees.I, II, III, IV degrees.- etiological:- etiological:primary, secondary, alimentary, cerebral, primary, secondary, alimentary, cerebral, endocrine.endocrine.

In obesity fat accumulates not In obesity fat accumulates not only in depot, it can be found in only in depot, it can be found in places where it never occurs in places where it never occurs in

healthy condition, for example in healthy condition, for example in myocardium, under pericardiummyocardium, under pericardium..

Heart in obesity, H&EHeart in obesity, H&E

Patients with obesity have Patients with obesity have highest risk development of:highest risk development of:

- atherosclerosis;- atherosclerosis;- arterial hypertension;- arterial hypertension;

- cholelythiasis;- cholelythiasis;- ischemic heart disease;- ischemic heart disease;

- diabetes mellitus;- diabetes mellitus;- liver steatosis- liver steatosis

Carbohydrate extracellular Carbohydrate extracellular accumulationsaccumulations

These degenerations are associated with These degenerations are associated with glycoproteins metabolism disturbances glycoproteins metabolism disturbances

and realize in tissues mucoidization.and realize in tissues mucoidization.Connective tissue, stroma of organs, fat Connective tissue, stroma of organs, fat

tissue, cartilages become swollen, tissue, cartilages become swollen, semitransparent, have mucus-like semitransparent, have mucus-like

appearance.appearance.Classic examples are mucoviscidosis Classic examples are mucoviscidosis

(hereditary), mixedema (insufficiency of (hereditary), mixedema (insufficiency of thyroid gland)thyroid gland)

Thank You for attention!Thank You for attention!