Kaposi's sarcoma - Causes, symptoms and treatment. MF.

  • Kaposi's sarcoma - Causes, symptoms and treatment. MF.

    In recent years, more and more scientifically substantiated reports about the connection of some oncological diseases with infectious pathogens appear. Kaposi's sarcoma is among them. Modern medicine can increasingly meet with such patients, especially because of the spread of the HIV pandemic.

    Historical background. For the first time Sarcoma Kaposi was described in 1872 by Austrian dermatologist Moritz Kohn from Kaposvar( also called Morits Kaposi).It was the name of the town that served as an example of the further name of the disease. During this period, the symptoms, visible changes on the skin, the morphology of the process are described. In Russia, the first case was described in 1883 by A.I.Pospelov. Since 1964, the first descriptions of cases of Kaposi's Sarcoma after organ and tissue transplantation on the background of immunosuppressive therapy have appeared, and since 1979, cases of Kaposi's Sarcoma in HIV-infected people under the age of 60 have become increasingly common. Since 1988, Kaposi's Sarcoma has been included in the list of AIDS-associated diseases in persons younger than 60 years. Since the same period, active viral influence in the onset of this disease has been proven.

    Kaposi's Sarcoma( CK) is a recurrent infectious tumor-like disease caused by herpesvirus type 8, which occurs against the background of immunodeficiency caused by genetic factors, the use of drugs( which have an immunosuppressive effect), and the human immunodeficiency virus, i.e.in patients with HIV infection.

    Herpes virus

    The problem of Kaposi's Sarcoma is becoming more urgent in connection with the AIDS pandemic. Often people from 20 to 40 years of age fall ill, often registered with people of non-traditional orientation( in recent decades - up to 13% of cases), acquires an epidemic spread( that is, in a number of patients it has been proved possible to transmit the disease, namely, the virus causing it).A particular problem is the complexity of early diagnosis, as well as in persons with immunodeficiency, the rapid spread of the disease and the defeat of internal organs. The high mortality and unfavorable prognosis of Kaposi's sarcoma is also a cause for concern.

    Causes of Kaposi's Sarcoma

    Depending on the causes, the following main forms of Kaposi's sarcoma are distinguished:
    1) The classic SC is a genetically predisposed Kaposi's sarcoma. Appears in persons over 60 years old with age-related immunodeficiency( may occur in family foci).
    2) African SC - endemic Kaposi's sarcoma, which can be caused by Epstein-Bar viruses, CMV, including herpes viruses of type 8.
    3) Epidemic SC - the main role is played by human immunodeficiency virus, as well as type 8 B-herpesvirus. That is, the role of the triggering factor is assigned to B-herpesvirus( HSV8) in conjunction with the human immunodeficiency virus( HIV) protein.
    4) Immunosuppressive SC - iatrogenic - the main role belongs to the development of pronounced deep immunodeficiency caused by the use of cytostatics and immunosuppressants( for example, in patients after organ and tissue transplantation).

    Symptoms of Kaposi's Sarcoma

    At the present stage of Kaposi's Sarcoma is a benign vascular tumor( endothelioma).The leading role in the formation of a malignant process is played by B-herpes viruses of type 8, which become active in conditions of a significant decrease in immunity.

    In the focus of Kaposi's sarcoma, the inner wall of the vessels( endothelium) proliferate, the structure of the vessels is broken( they become "like a sieve"), and specific spindle-shaped cells appear-this is the histological marker of Kaposi's sarcoma! Simultaneously with the blood vessels in Kaposi's sarcoma, lymphatic vessels are also affected.

    Localization of Kaposi's sarcoma is distinguished by:
    1) with localization on the skin of the feet, shins( spotted and small-knot formations), which is characterized by a chronic and benign course;
    2) with the predominance of edema of the feet, legs, thighs, brushes, in which the subacute and chronic course;
    3) base-palm version( nodular formations);
    4) visceral form - without skin lesion or with single elements on the skin, however with the defeat of internal organs, in which the forecast is often unfavorable.

    Kaposi's sarcoma on the hand

    SK of the rear

    According to the nature of the rash in Kaposi's sarcoma:
    1) Nodular SC - the appearance of cyanotic-brown or brownish-brown nodules, plaques on the skin of the lower extremities, and then everywhere( upper limbs, trunk, face);
    2) Red SK - the appearance of mushroom-like growths on the skin of red color resembling a polyp on the stem;
    3) Infiltrative SC - in addition to skin manifestations, there are lesions of such systems as muscular, bone;
    4) Disseminated SC - lymphadenopathy is a generalized SC( the process spreads with the damage of any system and organ).

    Skin nodule with Kaposi's sarcoma

    The main symptoms of

    sarcoma The most common symptom for this disease is in most cases:
    1) skin rash: may be spots, nodules, plaques, tumors, vesicles;the size of the element can be different from the millet grain to the pea;in color from red-cyanotic to dark-brown elements.
    With worsening of the condition of the nodular elements ulcers can form: deep with outlined somewhat twisted edges( like lips), blue-purple, with bloody necrotic touch and fetid detachable. In 1/3 of patients, hemorrhages( hemorrhages), patchy-purple rash and swelling of the tissues appear.
    Another common symptom of Kaposi's sarcoma is
    2) edema: edema may be primary( occur before the onset of the rash), and may be secondary( ie, appear simultaneously or after the appearance of the rash).Edema can occur on one limb, but can also be symmetrical. The skin acquires a reddish-cyanotic color, becomes dense, uneven. The patient has pain, heaviness in the affected limb, elephantiasis.
    The third common clinical symptom of Kaposi's Sarcoma is
    3) Limadenopathy: it always indicates the spread of the process and is characterized by an increase in one or more groups of lymph nodes.
    4) lesions of organs and systems , observed in the development of visceral forms of Kaposi's sarcoma( both with or without skin lesions).Affected by organs such as liver, gastrointestinal tract( ulceration, erosion), CNS, adrenal glands, bone tissue( destruction of bones, loss of calcium).Clinically, the damage to the internal organs is difficult to establish, because the symptoms of the disease do not differ from organ damage caused by other causes and it is difficult to diagnose Kaposi's sarcoma diagnostically without skin manifestations.
    5) rash on the mucous membranes ( mucous membranes of the mouth, cheeks, palate, tongue, pharynx, larynx, eyes) - small reddish-purple small nodules appear, which may increase in size and spread;- may appear crimson spots with a bluish tint isolated or together with nodular elements.
    6) there may be nonspecific complaints of ( pain in the affected limb, burning sensation, paresthesia - a feeling of crawling).

    SK solid skies

    Small intestine SK

    A brief description of the individual forms of Kaposi's Sarcoma

    I. Classical Kaposi's sarcoma( UK of the elderly).
    - occurs in people over 60 years old, against a background of chronic diseases( hypertension, atherosclerosis of the aorta, bronchitis, mycoses, gastritis, diabetes, psoriasis and others);
    - any part of the body is affected: more often n / extremities, the area of ​​the feet, shins over the joints, but m.on the head - ears, hairy part. Primary elements arise over the joints;
    - the symmetry of the rashes is characteristic;
    - the disease takes a long, chronic course - 8-10 years or more, and the state of health remains satisfactory for a long time, that is, benign flow.

    II.African SC( endemic) or subacute Kaposi's sarcoma.
    - occurs in persons younger than 35 years and in children of different age groups;
    - high frequency of visceral forms( involvement of internal organs) without skin manifestations;
    is a characteristic isolated lesion of lymph nodes without skin manifestations( cervical, axillary, inguinal), the younger the patient, the more pronounced the lymphadenopathy;
    - rapid progression of the disease with a lethal outcome within a few months;
    - the asymmetry of the rashes takes place;
    - there are such complaints as a feeling of heat, sweating, a general itch, throbbing pains - last a few months before the appearance of a local process.

    III.Epidemic SC( occurs in HIV-infected patients).
    - Age of patients under 40 years;
    - Start sharp, sudden;
    - More often primary localization on the upper part of the body( head, face, neck), and in 30% the process is localized in the oropharynx - the sky, gums, tongue, tonsils;
    - Characteristic: multifaceted lesions, asymmetry, bright red, crimson coloring of elements, polymorphism( different types of rash from spots to nodes);
    - Typically, the mucosal lesions, ulceration of elements;
    - Often the process is generalized or spread;
    - Lymphadenopathy is observed( all groups are enlarged - even intrathoracic, retroperitoneal lymph nodes);
    - The flow is aggressive, malignant, spontaneous resolution is practically absent.
    - Extremely unfavorable outcome - up to 80% of patients die by the 2nd year from the moment of their onset.

    Epidemic SC

    IV.Immunosuppressive SC.
    The most malignant form of Kaposi's sarcoma. Occurs when treated with immunosuppressants, cytostatics. It develops suddenly. There are many nodules that rapidly increase in size, turning into a tumor. The process quickly progresses, internal organs are affected. There is complete resistance( resistance) to treatment. The outlook is unfavorable.

    Diagnosis of Kaposi's sarcoma

    A preliminary diagnosis is made on the basis of socio-epidemiological data( suspicion of HIV infection, patient's age, presence of chronic diseases, previous organ transplantation, immunodeficiency) and clinical data( characteristic symptoms of some form of Kaposi's Sarcoma).If you suspect a HIV infection, a thorough diagnosis of this infection is carried out. If suspected involvement of internal organs and systems requires thorough instrumental diagnosis( ultrasound, FGD, sigmoidoscopy, bronchoscopy).

    The final diagnosis of Kaposi's Sarcoma is exhibited after histological examination of the study material( mucosal biopsy, skin and others).Histologically, the diagnosis is confirmed by the detection of spindle-shaped cells.

    Specific spindle-shaped cells in Kaposi's sarcoma

    When diagnosed, a number of similar diseases and symptoms should be excluded. These include:
    1. Pseudosarcoma - develops against a background of chronic venous insufficiency, the elements of the rash are painful, veins are observed, hemorrhagic spots, in the area of ​​spots one can listen to systolic noise, determine pulsation. There are no histologically spindle-shaped cells.
    2. Piogenic granuloma.
    3. Melanoma.
    4. Leiomyoma - is formed from the walls of small vessels, painful nodules are formed, more often unilateral, on the extensor surface. Symptom Turen - when frozen with chloroethyl nodules coagulate, shrink. In CS this symptom is negative.
    5. Angiolemiomy.
    6. Sarcoidosis Bénie-Beca-Shaumana is a large-nodular vascular tumor.
    7. Chromomycosis - deep mycosis, papillomatous growths, ulcers with scalloped edges.
    8. Pigmentary nevi is a papillomatous verruzed tumor.

    Treatment of Kaposi's sarcoma

    Treatment of single foci is performed surgically( excision of the focus) followed by radiotherapy. Such treatment with classical Kaposi's sarcoma leads to a successful result( long-term remission) in 30-40% of patients.

    If a patient with generalization of Kaposi's sarcoma, and in particular, in an HIV-infected person, a complex of antiretroviral therapy, chemotherapy, interferon therapy, and radiation therapy is shown( but in the stage of AIDS this often does not lead to the desired result).
    1. Highly Active Antiretroviral Therapy( HAART)
    - helps suppress viral load and increase immune status in HIV infection;
    - antiretroviral drugs can completely suppress the vital activity of one of the herpes viruses, which causes an oncological disease - Kaposi's sarcoma;
    - the duration of such therapy should be at least a year.
    2. Chemotherapy , with the aim of using prospidin( domestic drug), vincristine and vinblastine( rozevin), etoposide, taxol, doxirubicin, bleomycin and others. The drugs have pronounced side effects on the organs of hematopoiesis and others, which often requires the appointment of hormonal therapy( prednisolone, dexamethasone).
    3. Interferonotherapy.
    Purpose: As an immunomodulatory agent, interferon preparations are prescribed, namely:
    alpha interferon recombinant 2a and 2b( intron, roferon, reaferon) or native( vellferon) at doses of 5-10 million IU / day IM, n / klong courses.
    4. Local therapy includes: radiation therapy, cryotherapy, application of special gels( panretin), local chemotherapy.

    Drugs that are effective enough to treat infection caused by HHV-8 have not yet been found.

    Prevention of Kaposi's Sarcoma

    Preventive measures are reduced to HIV prevention and prevention of infection with type 8 herpesvirus. Also, preventive measures are taken after the diagnosis is made to prevent repeated manifestations of the disease, the progression of the process and the development of an unfavorable outcome.

    Doctor infectious disease Bykova N.I.