Liver amyloidosis
Contents:
Description:
Liver amyloidosis — manifestation of a general disease; it is observed along with damage of kidneys, adrenal glands, spleens, intestines and other bodies. Very seldom arises separately.
Liver amyloidosis reasons:
Development of an amyloidosis is connected with disturbance of proteinaceous and synthetic function of reticuloendothelial system, accumulation in a blood plasma of the abnormal proteins serving as autoantigens and causing formation of autoantibodies. Interaction of antigen with an antibody is resulted by sedimentation of the coarse-dispersion proteins participating in formation of amyloid. Being postponed in fabrics (for example, in walls of vessels, ferruterous, etc.), amyloid forces out functionally specialized elements of body that leads to death of this body.
Pathogeny:
In development of an amyloidosis the large role is played by immune disturbances, changes of protein metabolism. On modern representations, at least some types of amyloid are fabric deposits of fragments of immunoglobulins. In 1931 Magnus-Levy found a frequent combination of release of protein of Bens-Jones and adjournment of amyloid in patients with a myeloma. On the amino-acid list of squirrels of Bens-Jones almost matches amyloid. There are messages on a structural community of fragments of polypeptide chains of immunoglobulins and proteins of amyloid fibrilla at an amyloidosis. The evidence of major importance of cellular immune responses in an amyloidosis pathogeny is obtained.
In a pathogeny of primary amyloidosis the major role is played by disturbances of synthesis of immunoglobulins as a result of a plasmocyte mutation.
In formation of some kinds of amyloid an important role is played by system of a complement (S1, SZ and S4 fraction). Assume that cell-bound immune complexes participate in development of a reactive amyloidosis.
The liver is involved in pathological process at these three forms of an amyloidosis. At a reactive amyloidosis the liver is surprised almost always, at hereditary - in 50% of cases.
Surface of a liver usually smooth, pale brown, substance dense, wax-like. It is remarkable that the amyloidosis does not lead to cirrhosis.
According to V. V. Serov, I. A. Shamov (1977), initial adjournment of amyloid in perisinusoidalny space of Disse of the centers of segments, then gradual distribution on the periphery is characteristic of a reactive system amyloidosis. At the hereditary and connected with changes of immunocytes amyloidosis of adjournment of amyloid are most expressed in vessels and a stroma of portal paths.
Proceeding from amyloid localization, allocate three histologic types of a hepatic amyloidosis: The I type - intralobulyarny; The II type - periportal; The III type - perivascular, mixed.
Amyloid at intralobulyarny type squeezes and deforms hepatic beams that leads to their atrophy. Separate groups of hepatocytes are immured by amyloid and collapse. At II and III types of an amyloidosis of a liver hepatic segments are kept, amyloid is laid in vessels and a stroma of portal paths.
The most specific to amyloid are its anisotropy and a dichroism after coloring of Congo by red or red Sirius. Secondary fluorescence of amyloid in ultra-violet light after coloring by thioflavin T or S is very specific. Essential value for specification of localization of amyloid and extent of damage of hepatocytes has electronic microscopic examination.
Liver amyloidosis symptoms:
The clinical picture erased. The most frequent symptom is the gepatosplenomegaliya. The liver is dense, low-painful, sometimes increases only in a late stage of a disease. Jaundice is observed seldom, not intensive character. Intra hepatic obstructive jaundice against the background of primary amyloidosis of a liver is possible. In some cases the hemorrhagic syndrome can develop. The laboratory research reveals characteristic proteinaceous shifts, especially Alfa-2-globulinemiyu, immune disturbances, often hyperactivity of an alkaline phosphatase of serum, other functional trials are a little changed. The puncture biopsy of a liver has the greatest diagnostic value. Microscopic examination reveals at primary amyloidosis amyloid localization preferential under an endothelium and an argyrophil membrane of intra lobular arteries of a liver and periarterial fabrics, at secondary — in a hepatic parenchyma between beams and capillaries. Test with Congo red has the known diagnostic value. Diagnosis is promoted by simultaneous identification of damage of kidneys, intestines, gums, especially at a puncture biopsy of these bodies.
Diagnosis:
For identification of an amyloidosis such small clinical symptoms as dry, pale, porcelain skin, a macroglossia have the known value. Due to the frequent damage of kidneys reasonablly repeated research of urine which allows to find a proteinuria. The hepatolienal syndrome without the previous hepatic anamnesis also has to set the doctor thinking on a possible amyloidosis. For diagnosis the puncture biopsy of a liver has crucial importance. Amyloid is found in the form of the homogeneous masses which is painted Congo red in pink color.
The diagnosis becomes more reliable at simultaneous identification of amyloid in a liver, intestines, gums.
Liver biopsy
Treatment of an amyloidosis of a liver:
The crude liver, extracts and hydrolyzates of a liver are shown (Sireparum, прогепар, рипазон). At primary amyloidosis appoint drugs of a quinolinic row (delagil, plaquenil). Sanatorium treatment is not shown. The forecast is rather favorable, the liver failure arises seldom. The forecast remains heavy for the amyloidosis which developed at noninfectious diseases. Prevention of an amyloidosis consists in prevention, timely diagnosis and treatment of persistent infections. Working capacity is limited.
