Kaposi 's sarcoma - Causes, Symptoms and Treatment .MF .
In recent years, an increasing number of science-based messages about the connection of certain cancers with infectious pathogens.Kaposi's sarcoma is one of them.Modern medicine allows more often occur with these patients especially because of the spread of HIV infection.
History. first described by Kaposi's sarcoma in 1872. Austrian dermatovnerologom Moritz Kohn from the city of Kaposvar (also called Moritz Kaposi).That's the name of the town and serve as an example to further the name of the disease.During this period, the symptoms described in visible changes in the skin, the morphology process.In Russia, the first case described in 1883 by scientist AIPospelov.C 1964 appeared the first descriptions of cases of Kaposi's sarcoma after transplantation of organs and tissues in the immunosuppressive therapy, and cases of Kaposi's sarcoma in HIV-infected individuals younger than 60 years old are increasingly registered in 1979.Since 1988, Kaposi's sarcoma on the list of AIDS-related diseases in individu
Kaposi's sarcoma (KS) is a tumor recurrent infectious disease caused by the herpes virus type 8, occurring against the background of immunodeficiency caused by genetic factors, the use of medications (immunosuppressive effects rendering), as well as the human immunodeficiency virus, ie,in patients with HIV infection.
problem of Kaposi's sarcoma is becoming increasingly important due to the AIDS pandemic.Often sick person from 20 to 40 years, often recorded in those homosexual (in the last decade - to 13% of cases), acquires the epidemic spread (ie, a number of patients proved the possibility of transmission of the disease, namely the virus, it causes).A certain problem is the difficulty of early diagnosis, as well as in individuals with immunodeficiency rapid spread of disease and damage internal organs.A certain alarming and rather high mortality and poor prognosis of Kaposi's sarcoma.
Causes Kaposi's sarcoma
Depending on the reasons, the following basic forms of Kaposi's sarcoma:
1) Classical SK - is genetically predisposed Kaposi's sarcoma.It appears in persons over 60 years of age with immune deficiency (may occur in family foci).
2) African SK - endemic Kaposi's sarcoma, which may be the cause of Epstein-Bar virus, CMV, including herpes virus type 8.
3) Epidemic UK - a major role played by the human immunodeficiency virus, and herpes B-8 type.That is, the role of the trigger factor assigned B-herpesviruses (HSV8) together with a protein of human immunodeficiency virus (HIV).
4) Immunosuppressive SC - iatrogenic - the main role is played by the development of pronounced deep immunodeficiency caused by the use of cytotoxic drugs and immunosuppressants (eg, in patients after transplantation of organs and tissues).
symptoms of Kaposi's sarcoma
At the present stage Kaposi's sarcoma - a benign vascular tumor (endotelioma).The leading role in the formation of a malignant process play B-8 types of herpes viruses, which are active in a significant decrease in immunity.
The outbreak of Kaposi's sarcoma occurs proliferation of the inner walls of blood vessels (endothelium), impaired vascular structure (they become "like a sieve"), and there are specific spindle cells - a histological marker of Kaposi's sarcoma!Simultaneously with the blood vessels in Kaposi's sarcoma and lymphatic vessels are affected.
On localization of Kaposi's sarcoma release:
1) the localization of the skin of feet, legs (spotted melkouzelkovye and education), which is characterized by a chronic and benign course;
2) with a predominance of edema feet, legs, hips, hands, in which for sub-acute and chronic;
3) plantar-palmar option (nodules);
4) visceral form - without skin lesions or unit cells in the skin, but with the defeat of the internal organs, in which the most unfavorable prognosis.
Kaposi's sarcoma in the hand
SC rear brake
By the nature of the rash in Kaposi's sarcoma is isolated:
1) nodular SC - appearance of bluish-brown or brown-brown nodules, plaques on the skin of the lower limbs, and then everywhere (upper limbs, trunk, face);
2) Red SC - appearance of mushroom growths on the skin of red color, resembling a polyp on a stalk;
3) Infiltrative SC - in addition to skin manifestations appear defeat of systems such as muscle, bone;
4) Disseminated SC - limfadenopaticheskaya - a generalized SC (going to defeat the spread of any system and organ).
cutaneous nodule in Kaposi's sarcoma
main symptoms of Kaposi's sarcoma
very first symptom of this disease is in most cases:
1) skin rash: it can be spots, nodules, plaques, tumors,vesicles;element size can vary from a millet seed to a pea;color from bluish-red to dark brown elements.
When deterioration of nodular elements can form ulcers: a deep outlined several Turned edges (lips), blue-purple, with bloody necrotic plaque and fetid.In 1/3 patients appear hemorrhage (bleeding), mottled purpuroznaya rash and swelling of tissues.
Another common symptom of Kaposi's sarcoma - a
2) edema: edema may be primary (occur before the rash appears), and perhaps a secondary (ie, to appear at the same time or after the appearance of the rash).Swelling may occur at one extremity, and may be symmetrical.The skin becomes reddish-bluish color, it becomes dense, uneven.The patient, pain, heaviness in the affected limb, elephantiasis.
the third part of the clinical symptoms of Kaposi's sarcoma is
3) limadenopatiya: it always points to the distribution process and is characterized by an increase in one or more groups limfatichekih nodes.
4) defeat organs and systems observed in the development of visceral forms of Kaposi's sarcoma (a skin lesion, and without it).It affects the organs such as the liver, gastrointestinal tract (ulcers, erosions), central nervous system, adrenal glands, bone tissue (bone destruction, loss of calcium).Clinically, the defeat of the internal organs is difficult to determine, because the symptoms are no different from organ damage caused by other reasons and suspect Kaposi's sarcoma diagnostically difficult without cutaneous manifestations.
5) rash on the mucous membranes (mucous membrane of the mouth, cheeks, palate, tongue, pharynx, larynx, eyes) - there are small nodules with millet grain reddish-purple, which can increase in size and spread;- May appear purple spots with a bluish tint isolated or together with nodular elements.
6) can be non-specific complaints (pain in the affected limb, burning sensation, paresthesia - a feeling of pins and needles).
Brief characteristics of individual forms of Kaposi's sarcoma
I. Classical Kaposi's sarcoma (KS elderly).
- occurs in people older than 60 years, on the background of chronic diseases (hypertension, aortic atherosclerosis, bronchitis, fungal infections, gastritis, diabetes, psoriasis and others);
- affect any area of the body, most often n / limbs, the feet, lower legs over the knuckles, but might beon the head - ears, hairy part.Primary cells occur over joints;
- characterized by symmetrical rash;
- disease becomes protracted, chronic - 8-10 years or more, with feeling long remains satisfactory, that is benign course.
II.African Insurance (endemic), subacute, or Kaposi's sarcoma.
- occurs in people younger than 35 years and in children of different age groups;
- high incidence of visceral forms (internal organ) without skin manifestations;
- typical of isolated nodal involvement without cutaneous manifestations (cervical, axillary, inguinal), and the younger the patient, the more pronounced lymphadenopathy;
- rapid progression of the disease fatal in a few months;
- there is asymmetry of rash;
- holds such complaints as hot flashes, sweating, general itching, throbbing pain - lasts a few months before the local process.
III.Epidemic SC (occurs in HIV-infected patients).
- Age of patients up to 40 years;
- Getting a sharp, sudden;
- Most primary location on the upper part of the body (head, face, neck), and 30% of the process is localized in the oropharynx - the sky, gums, tongue, tonsils;
- Characteristic: multifocal lesions, asymmetry, bright red, purple coloring elements, polymorphism (different character rash of spots to nodes);
- characteristic lesions of the mucous membranes, ulceration of the elements;
- often occurs generalization or distribution process;
- There lymphadenopathy (increase all groups - even intraperitoneal, retroperitoneal lymph nodes);
- Current aggressive, malignant, spontaneous resolution is practically absent.
- Extremely poor outcome - up to 80% of patients dying in the 2nd year since the beginning.
most malignant form of Kaposi's sarcoma.Occurs when the treatment of immunosuppressants, cytostatics.Develops suddenly.You receive a lot of knots, which rapidly increase in size, becoming a tumor.The process is progressing rapidly, it affects the internal organs.There is a complete resistance (resistance) to the treatment.Prognosis is poor.
diagnosis of Kaposi's sarcoma
preliminary diagnosis is based on the social and epidemiological data (suspected HIV infection, the 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 HIV infection shall provide a thorough diagnosis of the infection.If you suspect damage internal organs and systems requires careful instrumental diagnostics (ultrasound, EGD, sigmoidoscopy, bronchoscopy).
final diagnosis of Kaposi's sarcoma is exposed after histological examination of the material (mucosal biopsy, skin, etc.).Histologically, the diagnosis is confirmed by detection of spindle-shaped cells.
specific spindle cells in Kaposi's sarcoma
When the diagnosis should be ruled out for a number of similar symptoms and manifestations of disease.These include:
1. Psevdosarkoma - develops on the background of chronic venous insufficiency, rash, painful elements, there is dilatation, hemorrhagic spots, spots in the area, you can hear a systolic murmur, determine the pulsation.Histologically, spindle-shaped cells do not have.
2. pyogenic granulomas.
4. Leiomyoma - formed from the small vessel walls, forming painful nodules, often one-sided, on the extensor surfaces.Symptom Touraine - freeze-chloroethyl nodules coagulate, cut.In the UK, this symptom is negative.
6. Sarcoidosis Besnier-Boeck-Schaumann - SKD vascular tumor.
7. chromomycosis - deep mycosis papillomatous growths, ulcers scalloped edges.
8. pigmented nevi - papillomatous verrucous tumor.
Treatment of Kaposi's sarcoma Treatment
individual foci is performed surgically (resection hearth) followed by radiotherapy.Such treatment of classical Kaposi's sarcoma leads to a successful outcome (long-term remission) in 30-40% of patients.
If a patient with a generalization of Kaposi's sarcoma, and in particular in HIV-infected, it shows a complex antiretroviral therapy, chemotherapy, interferon therapy, radiation therapy (however in the stage of AIDS is often not lead to the desired result).
1. Highly antiretrovirusnyya therapy (HAART)
- contributes to the suppression of viral load and improve immune status in HIV infection;
- antiretroviral drugs can completely suppress the vital activity of one of the herpes viruses that cause cancer - Kaposi's sarcoma;
- the duration of such therapy should be at least one year.
2. Chemotherapy , with the aim of which is used prospidin (domestic product), vincristine and vinblastine (rozevin), etoposide, taxol, doxirubicin, bleomycin and others.The drugs have strong side effects on the organs of blood formation and others, which often requires the appointment of hormone replacement therapy (prednisolone, dexamethasone).
Objective: as immunomodulatory effects of interferon administered drugs, namely:
recombinant interferon alfa-2a and 2b (Intron, Roferon, reaferon) or native (vellferon) at doses of 5-10 million IU / day / m n /for long courses.
4. Local therapy includes: radiation therapy, cryotherapy, application of special gels (panretin), local chemotherapy.
Drugs that would be sufficiently effective in treating infections caused by HHV-8, has not been found.
Prevention of Kaposi's sarcoma
Preventive measures to reduce HIV infection and prevention of herpesvirus type 8.Also preventive measures are carried out after the diagnosis to prevent recurrence of disease, disease progression and the development of adverse outcome.
infectious disease doctor Bykov NI