3. Amyloid consists of two components with antigenic properties:

a) The P component (plasma) - plasma glycoproteins

b) the F-component (fibrillar) is a heterogeneous, four varieties of the F-component:

/1. AA-protein - is not associated with Ig - from serum -globulin SSA

2. AL-protein - associated with Ig - from the Ig- and Ig-light chains

3. FAP protein is formed from prealbumin

4. ASC 1-protein - is formed from prealbumin

Structure: Amyloid consists of 4 main components: fibrillar protein (F-component), periodic rods (P-component), glycosaminoglycans and so-called "hematogenic additives".

Stages of morphogenesis of amyloidosis:

1. Transformation of cells with the formation of amyloidoblasts

2. Synthesis of fibrillar protein in amyloidoblasts

3. Aggregation of fibrils outside cells (i.e., amyloidoblasts produce fibrils, then the fibrils exit into the intercellular substance without damaging the amyloidoblast)

4. The connection of fibrils with plasma proteins and chondroitin sulfate

Methods for detecting amyloid (reactions are based on the phenomenon of metachromasia):

1. Congo-red coloring - in red

2. coloring with Lugol's solution with 10% sulfuric acid solution - in blue

3. coloring with methyl violet - in red

4. dichroism and anisotropy in a polarization microscope

4) Classification of amyloidosis:

1. By reason (origin):

1. idiopathic primary (AL-amyloidosis)

2. Hereditary (genetic, family): a. periodic disease (familial Mediterranean fever) b. Macle-Wales syndrome (a and b - AA-amyloidosis) c. familial amyloid polyneuropathy (FAP-amyloidosis)

3. secondary acquired: a. reactive (AA-amyloidosis in chronic infections, CKD, osteomyelitis, wound suppuration, rheumatoid arthritis) b. monoclonal protein (AL-amyloidosis in paraproteinemic leukemia)

4. Senile systemic amyloidosis (ASC1-amyloidosis) and local

2. according to the specifics of the fibrill protein:

1. AL- (generalized damage to the heart, lungs, blood vessels)

2. AA- (generalized damage mainly to the kidneys)

3. FAP- (damage to peripheral nerves)

4. ASC1- (mainly damage to the heart and blood vessels)

3. by prevalence:

1. generalized: primary, secondary, systemic senile

2. local: forms of hereditary amyloidosis, senile local amyloidosis, "amyloid tumor"

4. by clinical manifestations:

1. cardiopathic

2. Epinephropathic

3. nephropathic

4. Neuropathic

5. APUD-amyloidosis

6. Hepatopathic

5) According to the localization of the lesion, amyloidosis is isolated:

1. perireticular ("parenchymal") - amyloid prolapse along the reticular fibers of the membranes of blood vessels and glands, the reticular stroma of the parenchyma (spleen, liver, kidneys, adrenal glands, intestines, intima of small and medium vessels)

2. Pericollagen ("mesenchymal") - amyloid prolapse along the collagen fibers of the adventitia of medium and large vessels, myocardium, striated muscles, MMC, nerves, skin.

14. Primary amyloidosis: causes, chemical structure of amyloid, mechanisms of development, examples of diseases. Macro- and microscopic changes, outcomes, clinical significance.

AL (primary)- type of amyloid protein

Light chains of immunoglobulins are the main source

Primary amyloidosis Plasma cell myeloma B-cell malignant lymphoma - diseases

Tongue, heart, gastrointestinal tract, liver, spleen, kidneys - localization

Macro- and microscopic characteristics of amyloidosis. The appearance of organs in amyloidosis depends on the degree of development of the process. If the amyloid deposits are small, the appearance of the organ changes little and amyloidosis is diagnosed only by microscopic examination.

• With severe amyloidosis, the organs increase in volume, pale, with a greasy sheen (hepatosplenomegaly, cardiomegaly, thickening of peripheral nerves, macroglossia). Damaged tissues have a denser consistency and reduced elasticity compared to normal tissues. The signs of the most pronounced tissue lesion are a pale gray shade and a peculiar waxy, or greasy, appearance of it on the incision.

Issue. Unfavorable, almost irreversible.

The functional significance is determined by the degree of development of amyloidosis. Pronounced amyloidosis leads to dystrophy and atrophy of the parenchyma and sclerosis of the stroma of organs, to their functional insufficiency. With severe amyloidosis, chronic renal insufficiency is most often observed, less often — hepatic, cardiac, pulmonary, adrenal, intestinal (malabsorption syndrome) insufficiency.