Kaposi's sarcoma is 85% of all tumors developing in AIDS patients. This multifocal malignant tumor of vascular origin, which affects the skin, mucous membranes and internal organs. She has several varieties, one of which is the epidemic's sarcoma associated with AIDS.
Tumor affects people under the age of 35 - 40 years old, is most common in passive homosexuals manifested spots, nodules or plaque bright red or reddish-brown in color, rapidly distributed to all of the skin, mucous membranes and internal authorities. over time the tumor elements merge to form tumor-like formations that eventually ulcerate. Kaposi's sarcoma is difficult to treat patients and quickly leads to death. Correct diagnosis is easily confirmed by the study of a piece of tissue under a microscope.
What it is?
Kaposi's sarcoma (angiosarcoma hemorrhagic sarcoma, or multiple sarcomatosis) is a multiple malignancies dermis (skin). First described by the Hungarian dermatologist Moritz Kaposi and named after him. The prevalence of the disease generally small, but Kaposi's sarcoma ranks first among malignant tumors affecting patients with HIV infection, reaching numbers 40-60%.
Causes of
It is for certain causes of occurrence of such tumors, are not known. But scientists are more likely to suggest that the disease can develop on the background of the human herpes virus type-8, which in itself is not yet sufficiently understood.
Kaposi's sarcoma is also often accompanied by other malignant processes, including:
- lymphosarcoma;
- multiple myeloma;
- mycosis fungoides;
- Hodgkin's lymphoma (megakaryoblastoma);
- leukemias.
For the occurrence of pathology must be a significant reduction in the human immune system due to various reasons. In addition, some groups of people the risk of Kaposi's sarcoma is much higher than the rest. For example, often the disease occurs in men than in women.
At risk are:
- HIV-infected persons;
- Face transplant donor organs (especially kidneys);
- elderly men belonging to the Mediterranean race;
- persons native to equatorial Africa.
Medical scientists agree on one thing: often, especially in the early stages of development, the disease is more likely is a reactive process (ie, occurring in response to an infectious disease) than true sarcoma.
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morbid anatomy
Typically, the tumor has a purple color, but the color may be different shades of red, purple or brown. The tumor may be flat or slightly above the wheel is a painless spots or nodules. Almost always located in the skin, at least - on the internal organs. Kaposi's sarcoma is often associated with damage to the mucous membrane of the palate, lymph nodes. During a slow disease. Detection of Kaposi's Sarcoma in HIV infection gives the basis for the diagnosis of AIDS.
Histology Tumor structure is characterized by a plurality of randomly arranged thin-walled newly-formed vessels and spindle cell bundles. Typical tumor infiltration by lymphocytes and macrophages. The vascular nature of the tumor dramatically increases the risk of bleeding. However, to do a biopsy for suspected Kaposi's sarcoma is not necessary. Kaposi's sarcoma - a special type of tumor, which often requires not only the verification of the diagnosis, but also its treatment. This may seem strange at first glance. This is due to the fact that the diagnosis can put infallible and without biopsy, and the isolated treatment of Kaposi's sarcoma rarely gives a complete healing.
Moreover, treatment of Kaposi's sarcoma (due to its connection with the main causative agents of disease) usually is palliative, i.e. directed only at reduction of symptoms.
symptoms
The clinical picture of Kaposi's Sarcoma is varied and depends on the duration of the disease.
In the initial stages sarcoma symptoms of various contours and dimensions is reddish-bluish spots and pink uzelkovopodobnye elements, which then become bluish color. As the flow of Kaposi's sarcoma lesions take the form of various sized nodular elements infiltrated reddish-bluish color. Nodularity data tend to merge, resulting in a large hilly foci formed with sharply painful ulcers. In the sealed chamber integuments, edematous, bluish-purple color. Mainly localized lesions on the skin distal extremities (94% of the lower limbs - the anterolateral surface of the tibia and feet) and tend to be located near superficial veins. Frequently observed symmetry of the affected limb.
At its downstream tumor may be sub-acute, acute and chronic. Acute for Kaposi sarcoma is characterized by rapidly progressing symptoms manifested generalized skin lesions in the form of a plurality of nodular formations on the body, face and limbs, and fever. These symptoms are accompanied by visceral and / or lymph nodes. Duration course of the acute form of two months to two years.
When subacute sarcoma skin rash generalization occurs much less frequently. Chronic characterized by gradual progression in the form of skin rashes and patchy nodular elements mottled. The duration of the chronic form of eight years or more.
Immuno-suppressive type
The disease occurs when receiving immunosuppressive drugs (usually after kidney transplantation). characterized by:
- scarce symptoms;
- chronic and benign course;
- rare involvement in the process of internal organs and lymph nodes.
When canceling the immunosuppressive drug is often marked regression of the disease.
epidemic type
The emergence of this new formation - one of the main symptoms of HIV infection. For such Kaposi sarcoma is characterized by:
- occurrence at a young age (before 40 years);
- neoplasms bright colors;
- characterized by mandatory involvement in the process of the mucous membranes;
- unusual layout sarcoma: tip of the nose, the mouth on the hard palate, upper extremities.
This type of disease is characterized by rapid and malignant course with involvement in the process of lymph nodes and internal organs.
endemic type
Occurs mostly in people in Africa, in Russia are not usually found. The distinctive features of this type are:
- usually occurs in childhood, in the first year of life;
- Characteristic lesions of lymph nodes and internal organs;
- skin pathological elements hardly occur.
current options
- Acute: rapid progression of the process, an unfavorable outcome without treatment comes from 2 months to 2 years from the onset of the disease.
- Subacute: in the absence of treatment, patients can live up to 3 years.
- Chronic: benign, in which patients can do without therapy 10 years or more.
What is Kaposi's sarcoma: photo
The photo below shows how the disease manifests itself in humans.
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Diagnostics
Diagnosis sarcomatosis sarcoma held in infectious diseases, dermatologist and oncologist. First, doctors listen to patient history and collected, and then:
- Check the signs of the disease.
- Do a biopsy.
- Conduct histological examination to detect the proliferation of fibroblasts.
- Conduct immunological studies.
- Take blood for HIV test.
Also, the patient is prescribed additional studies, such as ultrasound, X-ray, gastroscopy, CT scan of the kidneys, adrenal glands and other MRI for the detection of internal injuries.
complications
Kaposi's sarcoma occurrence of complications depend on the stage of the disease and the tumor site. May cause the following complications:
- lymphatic edema, elephantiasis due kinked lymph nodes;
- bacterial infection damaged tumors;
- restriction of motor activity of the limbs and their deformation;
- bleeding from decaying tumors;
- intoxication caused by the collapse of neoplasms;
- disruption of the internal organs in the localization of tumors on them.
Some complications lead to a life-threatening condition of the person.
Treatment of Kaposi's Sarcoma
Treatment for single foci surgically (excision hearth) followed by radiotherapy. Such treatment of classical Kaposi's sarcoma leads to a successful outcome (prolonged remission) in 30-40% of patients.
If a patient with Kaposi's sarcoma generalization, and in particular, HIV-infected, the complex shows antiretroviral therapy, chemotherapy, interferon therapy, radiation therapy (however, in the stage of AIDS is not usually lead to the desired result).
1) Highly antiretrovirusnyya therapy (HAART)
- the duration of such therapy should be at least one year;
- It contributes to the suppression of viral load and enhancing the immune status of HIV-infection;
- antiretroviral drugs can completely suppress the vital activity of one of the herpes viruses that cause cancer - Kaposi's sarcoma.
2) chemotherapy, which are used for the purpose prospidin (domestic product), vincristine and vinblastine (rozevin), etoposide, taxol, doxirubicin, bleomycin and others. Drugs have severe side effects on the organs of blood formation and others, which often requires the appointment of hormone replacement therapy (prednisone, dexamethasone).
3) interferon.
- Purpose: as an immunomodulating action prescribed drugs interferon, namely interferon alpha recombinant 2a and 2b (Intron, Roferon, reaferon) or native (vellferon) at doses of 5-10 MIU / day / m, n / k long courses.
4) Local therapy includes: radiation therapy, cryotherapy, application of special gels (panretin), local chemotherapy.
Drugs, which would be sufficiently effective in the treatment of infections caused by HHV-8, has not been found.
Prediction of Kaposi's sarcoma
Prognosis in Kaposi's sarcoma depends on the character of its flow and is closely related with the state of the patient's immune system.
At higher rates of immunity manifestations of the disease can be worn reversible, systemic treatment gives a good effect, and can achieve remission in 50-70% of patients. Thus in patients with Kaposi's sarcoma CD4 lymphocytes index greater than 400 l-1 on the background response rate ongoing immune therapy exceeds 45%, and with less than 200 CD4 l-1 is possible only to achieve remission 7% patients.
prevention
The main measure of disease prevention is the timely and correct treatment of immunodeficiency states. Thus, the use of antiretroviral drugs in HIV-infected patients allows a long time to maintain normal immune system function, thereby preventing the occurrence of Kaposi's sarcoma.
After treatment of Kaposi's sarcoma requires careful examination of the skin and mucous membrane at least once in 3 months, the evaluation of lung conditions and gastrointestinal tract - at least once in six months or a year. These measures will help to timely detect disease recurrence.
