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Kaposi's sarcoma - a rare cancer

Kaposi's sarcoma in HIV-infected patients is among the "AIDS-indicator" diseases. Detection of a tumor of this type in young people without obvious impairment of the immune system provides a basis for making a diagnosis of HIV infection in the AIDS stage, even without using laboratory research methods. Kaposi's sarcoma accounts for 85% of all tumors that develop in AIDS patients. This is a malignant multifocal tumor of vascular origin, which affects the skin, mucous membranes and internal organs. It has several varieties, one of which is the AIDS-related sarcoma.

The tumor affects people under the age of 35 - 40 years, most often occurs in passive homosexuals, manifested by spots, nodules or plaques of a bright red or red-brown color, spreading rapidly throughout the skin, mucous membranes and internal organs. Elements of the tumor eventually merge to form tumor-like formations, which eventually ulcerate. Kaposi's sarcoma is difficult to treat and quickly leads patients to death. The correct diagnosis is established easily and confirmed by examining a piece of tissue under a microscope.

What it is?

Kaposi's sarcoma (Kaposi’s angiosarcoma or multiple hemorrhagic sarcomatosis) is a multiple malignant neoplasm of the dermis (skin). First described by the Hungarian dermatologist Moritz Kaposi and named after him. The prevalence of this disease is generally small, but Kaposi’s sarcoma is the first among malignant neoplasms affecting HIV patients, reaching 40–60%.

Causes

It is not known for certain reasons causing the appearance of such neoplasms. But scientists are very likely to suggest that the disease may develop against the background of the human herpes virus of the 8th type, which in itself has not yet been sufficiently studied.

Also often, Kaposi's sarcoma accompanies other malignant processes, including:

For the emergence of pathology requires a significant reduction in human immunity due to various reasons. In addition, some groups of people have a much higher risk of Kaposi's sarcoma than the rest. For example, more often the disease occurs in men than in women.

In the risk group are:

  • HIV-infected individuals
  • persons with organs transplanted from donors (especially kidney),
  • elderly men of the Mediterranean race
  • persons whose homeland is equatorial Africa.

Medical scientists are unanimous in one thing: most often, especially in the initial stages of development, this disease is more likely a reactive process (that is, arising in response to an infectious lesion) than true sarcoma.

Pathological anatomy

Usually, the tumor has a purple color, but the color may have different shades: red, purple or brown. The tumor may be flat or slightly rise above the skin, is a painless spots or nodules. Almost always located on the skin, at least - on the internal organs. Kaposi's sarcoma is often combined with damage to the mucous membrane of the palate, lymph nodes. The course of the disease is slow. Detection of Kaposi's sarcoma in HIV infection provides the basis for the diagnosis of AIDS.

The histological structure of the tumor is characterized by a multitude of chaotically located thin-walled newly formed vessels and bundles of spindle-shaped cells. The tumor is infiltrated with lymphocytes and macrophages. The vascular nature of the tumor dramatically increases the risk of bleeding. However, biopsy for suspected Kaposi's sarcoma is not necessary. Kaposi's sarcoma is a special type of tumor, which often does not require not only verification of the diagnosis, but also its treatment. This may seem strange at first glance. This situation is due to the fact that an accurate diagnosis can be made without a biopsy, and the isolated treatment of Kaposi's sarcoma extremely rarely gives complete healing.

Moreover, the treatment of Kaposi's sarcoma (due to its connection with the causative factors of the underlying disease) is usually palliative, that is, only aimed at reducing the symptoms of the disease.

The clinical picture of Kaposi's sarcoma is quite diverse and depends on the duration of the disease.

In the initial stages of the symptoms of sarcoma, reddish-bluish spots of various shapes and sizes appear, as well as pink nodules, which then turn bluish in color. As the Kaposi's sarcoma progresses, the eruptions take on the appearance of nodular infiltrated elements of a reddish-bluish color, different in size. These nodules tend to merge, which leads to the formation of large nodular foci with formed sharply painful ulcers. In the area of ​​the focus of the skin compacted, swollen, purple-bluish color. Mainly foci are localized on the skin of the distal extremities (94% on the lower extremities - anterolateral surfaces of the legs and feet) and tend to be located near the superficial veins. Often marked symmetry of the lesions of the limbs.

According to its course, the tumor can be subacute, acute and chronic. The acute course of Kaposi's sarcoma is characterized by rapidly progressive symptoms and is manifested by generalized skin lesions in the form of a multitude of nodular lesions on the body, face and limbs, as well as fever. These symptoms are accompanied by damage to the internal organs and / or lymph nodes. The duration of the acute form from two months to two years. In subacute sarcoma, generalization of skin lesions is less common. Chronic course is characterized by gradual progression of skin rashes in the form of plaque and spotty-nodular elements. The duration of the chronic form of eight years or more.

Immune suppressive type

The disease occurs when taking 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.

With the abolition of the immunosuppressive drug is often marked regression of the disease.

Epidemic type

The occurrence of this neoplasm is one of the main symptoms of HIV infection. For such Kaposi's sarcoma is typical:

  • occurrence at a young age (up to 40 years),
  • neoplasms of bright color,
  • characteristic mandatory involvement in the process of mucous membranes,
  • unusual arrangement of elements of sarcoma: at the tip of the nose, in the mouth on a hard palate, upper limbs.

This type of disease is characterized by a rapid and malignant course involving lymph nodes and internal organs in the process.

Endemic type

Occurs mainly in Africans, in Russia usually does not occur. Distinctive features of this type are:

  • usually occurs in childhood, in the first year of life,
  • characterized by damage to the lymph nodes and internal organs,
  • on the skin pathological elements practically does not occur.

Flow options

  • Acute: rapid progression of the process, unfavorable outcome without treatment occurs from 2 months to 2 years from the onset of the disease.
  • Subacute: in the absence of therapy, patients can live up to 3 years.
  • Chronic: a benign course in which patients can do without therapy for 10 years or more.

Diagnostics

Diagnosis of Kaposi's sarcomatosis is carried out by an infectious diseases specialist, dermatologist and oncologist. At first, the doctors listen to the patient and take a history, and then:

  1. Check the signs of the disease.
  2. Do a biopsy.
  3. Conduct a histological study to identify the proliferation of fibroblasts.
  4. Conduct immunological studies.
  5. Blood is taken for analysis of HIV infection.

Also, the patient is prescribed additional studies, such as ultrasound, radiography, gastroscopy, CT scan of the kidneys, MRI of the adrenal glands and others to detect internal lesions.

Complications

The occurrence of complications of Kaposi's sarcoma depends on the stage of development of the disease and the localization of tumors. The following complications may occur:

  • lymphatic edema, elephantias due to lymph node squeezing,
  • bacterial infection of damaged tumors,
  • limitation of motor activity of the extremities and their deformation,
  • bleeding from disintegrating tumors,
  • intoxication caused by the disintegration of tumors,
  • violation of the internal organs during the localization of neoplasms on them.

Some complications lead to life-threatening conditions.

Treatment of Kaposi's Sarcoma

Treatment of single lesions is performed surgically (excision of the lesion), followed by radiation therapy. 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 for an HIV-infected person, then a complex of antiretroviral therapy, chemotherapy, interferon therapy, radiation therapy is shown (however, this often does not lead to the desired result in the AIDS stage).

1) Highly Active Antiretroviral Therapy (HAART)

  • the duration of such therapy should be at least a year
  • contributes to the suppression of viral load and increase the immune status of HIV infection,
  • Antiretroviral drugs can completely suppress the vital activity of one of the herpes viruses that causes cancer — Kaposi’s sarcoma.

2) Chemotherapy, for the purpose of which prospidin (a domestic drug), vincristine and vinblastine (rozevin), etoposide, taxol, doxirubicin, bleomycin and others are used. The drugs have pronounced side effects on the blood-forming organs and others, which often requires the appointment of hormonal therapy (prednisone, dexamethasone).

  • Purpose: as an immunomodulating effect, interferon preparations are prescribed, namely: alpha interferon recombinant 2a and 2b (intron, roferon, reaferon) or native (wellferon) in doses of 5-10 million IU / day w / m, s / c long courses .

4) Local therapy includes: radiation therapy, cryotherapy, application of special gels (panretin), local chemotherapy.

Drugs that would be quite effective in treating HHV-8 infection have not yet been found.

Prognosis of Kaposi's sarcoma

The prognosis of the disease in Kaposi’s sarcoma depends on the nature of its course and is closely related to the state of the patient’s immune system. With higher immunity, manifestations of the disease can be reversible, systemic treatment gives a good effect and allows to achieve remission in 50-70% of patients. So, in patients with Kaposi's sarcoma with a CD4 lymphocyte count of more than 400 μl-1, the frequency of remission against the background of ongoing immune therapy exceeds 45%, and with CD4 less than 200 μl-1, only 7% of patients can achieve remission.

Prevention

The main measure of prevention of the disease is timely and proper treatment of immunodeficiency states. Thus, the use of antiretroviral drugs in HIV-infected patients allows for a long time to maintain normal function of the immune system, thereby preventing the appearance of Kaposi's sarcoma.

After the treatment of Kaposi's sarcoma, a thorough examination of the skin and mucous membranes is necessary at least once every 3 months, the assessment of the condition of the lungs and the gastrointestinal tract - at least once every six months or a year. These measures will help in time to identify the recurrence of the disease.

What is Kaposi's sarcoma?

Kaposi's sarcoma (hereinafter SC) is a cancer that causes spots on the skin, in the mouth, nose and throat, in the lymph nodes or other organs, including the lungs and esophagus. These spots, or lesions, are usually red or purple. They are formed from cancer cells of blood vessels and blood cells.

Skin lesions of Kaposi's sarcoma most often appear on the legs or on the face. They may look bad, but do not cause any symptoms. Only some lesions on the legs or groin can cause painful swelling of the legs and feet.

SK can cause serious problems or even become life threatening when the lesions are in the lungs, liver or in the digestive tract. Tumors in the digestive tract, for example, can cause bleeding, while tumors in the lungs make breathing difficult.

In the photo: Kaposi's sarcoma. Characteristic violet plaques on the wings and tip of the nose in an HIV-infected patient.

The disease was named after the dermatologist Moritz Kaposi, who first described it.

Epidemiology of the disease

In HIV-infected individuals, Kaposi's sarcoma occurs 300 times more often than in patients taking immunosuppressive therapy, and 2,000 times more often than among the general population. In the initial period of the pandemic (from 1980 to 1990), the disease occurred in 40–50% of AIDS patients (mainly in homosexuals and bisexuals). With the use of combination highly active antiretroviral therapy, especially since the use of protease inhibitors, there has been a decrease in the number of patients to 9–18%. In women with acquired immunodeficiency syndrome, the tumor develops in case of sexual contact with bisexual partners.

Fig. 2. Kaposi's sarcoma in an HIV patient on the genitals.

Fig. 3. Kaposi's sarcoma on the palms. The initial stage (photo on the left) and the tumor stage (photo on the right).

Development factors

The disease develops due to the ingestion of the herpes virus type 8 against the background of reduced immunity.

Provoke the occurrence of angiosarcoma:

  1. HIV infection, since on the background of immunodeficiency, tumor cells "do not interfere" with the development of the body's defenses,
  2. conditions in which immunity is artificially suppressed - for example, chemotherapy for cancer, medication after a successful organ transplant,
  3. age characteristics of the body's defenses in childhood and old age,
  4. genetic factors - people in Africa and the Mediterranean countries, especially males, are more prone to the disease of multiple hemorrhagic sarcomatosis.

The disease is often found in men, especially older people are prone to it. In HIV-infected patients, occurs in more than half of cases.

Classification

Kaposi's sarcoma in the ICD 10 (the international classification of diseases of the 10th revision) stands at number C46, ​​in the block of malignant neoplasms of soft tissues of the “Neoplasm” class.

In the list there are “subtypes” of the disease depending on the location - for example, skin lesions will be designated as C46.0, and internal organs - C46.8.

According to the factors that provoked the disease, emit:

  • the classical type is genetically predisposed, it can be family-like, it is found on the territory of Russia and Europe,
  • endemic or African - affects mainly young children, characterized by the absence or small number of skin manifestations - spots, nodules and others,
  • epidemic - Kaposi's sarcoma in HIV, is characterized by a malignant, bright course with unusual symptoms - for example, it can manifest itself in the oral cavity (particularly in the sky),
  • iatrogenic, immune suppressive or medicinal - against the background of therapy, the most benign of all (often passes on its own after the abolition of drugs that provoked the disease).

Downstream herpetic eczema can be:

  • acute form - when a person dies without treatment after a period of 2 months to 2 years after the onset of the disease,
  • Subacute - up to 3 years
  • chronic - benign, the patient can live up to 10 years or more even without the help of doctors.

By localization and the nature of external manifestations are distinguished:

  • nodular - skin is covered with brown-brown and bluish formations, plaques. The favorite place of appearance is the soles of the feet and the palms,
  • red - the appearance of growths, resembling a polyp on a leg or a mushroom,
  • infiltrative - it occurs with a lesion not only of the skin, but also of the muscles, bones,
  • disseminated or generalized - the disease covers the entire body, and skin lesions may not be at all.

Depending on the type, the symptoms of Kaposi's sarcoma may vary somewhat, but are generally characterized by:

  • skin manifestations in the form of rashes, spots, nodes, tumors, plaques of various sizes - from tiny to diffuse,
  • variegated colors - the elements are red-bluish, red-brown, dark brown, blue-purple,
  • the presence of hemorrhages, hemorrhages,
  • pathological changes of the mucous membranes of the oral cavity, similar to the skin,
  • swelling - may appear before the skin lesions, and may simultaneously with them (the limb becomes “heavy”, does not leave a feeling of squeezing, a bluish tinge appears, pain may join),
  • swollen lymph nodes - one or a group
  • systemic damage to the organs, characteristic of severe course (ulcers and erosion appear in the gastrointestinal tract (GAL and duodenum, for example), the central nervous system, bone tissue).

To establish the true cause of such violations is sometimes extremely difficult.

Classic

Классический тип болезни протекает в три стадии:

  1. spotty, initial stage of Kaposi's sarcoma - flat elements are formed on the skin of various colors of irregular shape,
  2. papular - when pathological changes become more voluminous, tighter to the touch, increase, can merge with neighboring formations,
  3. tumor - large nodes of various consistency are formed.

Occurs in the elderly against the background of age-related changes in the body, comorbidities (hypertension, diabetes and other diseases).

The rash is symmetrical, often the first occur in the periarticular areas, on the leg, and then “go away” on the feet, legs, sometimes the scalp. For a long time, well-being remains acceptable.

African

The African type is aimed mainly at the internal organs - skin manifestations may not be at all. Young people under 35 suffer from the disease, and more often - babies under one year old. The rash is chaotic, the lymph nodes are greatly affected.

The general condition suffers - the patient may note:

The development of acute, fatal outcome may occur already during the first months of the disease.

Endemic

The endemic type, or Kaposi's sarcoma in HIV-infected individuals, begins acutely and develops malignantly. For the first time, rashes appear in the mouth - the gums, tongue and tonsils are affected, ulcers and erosion develop.

A rash appears on the mucous membrane of the sky. Pathological lesions look unusual, for example, can be a red spot on the lip. Lymph nodes are enlarged, the disease often becomes generalized, the most aggressive and evil in nature.

Iatrogenic

Iatrogenic type is characterized by a chronic favorable course, can sometimes be cured on its own after discontinuation of drugs - “provocateurs” of the disease. Such a complication of drug therapy does not frighten doctors at all - it is the result of serious treatment of serious diseases, which in themselves can be fatal much faster than Kaposi’s angioreticulosis.

Traditional medicine

Herpetic eczema can only be cured with the help of official medicine. Therapy is carried out under the watchful eye of a specialist.

Local therapy is used to correct the visible manifestations of the disease - for example, reducing the size of tumor elements or even eliminating them. Apply:

  • cryotherapy, treatment of Kaposi's sarcoma with cold,
  • radiation methods to eliminate cosmetic defect,
  • injection into the tumor - for example, vinbalstin, (antitumor drug),
  • medications in the form of applications.

Occasionally, complications develop in the form of open infected ulcers — then antibiotics can be used systemically or topically.

General therapy is used in extreme cases, because it has a devastating effect on the bone marrow. There are strict indications for systemic treatment:

  • lesions of internal organs
  • low effect of local therapy,
  • weight loss of more than 10%.

  • in people with HIV infection, first and foremost, the emphasis is on restoring the body's defenses - anti-retroviral therapy is prescribed to fight the pathogen,
  • chemotherapy of Kaposi's sarcoma is carried out with the rapid progression of the disease, its generalization (resorted to the method with the defeat of the internal organs),
  • immunomodulating agents, activating the body's defenses - interferons.

Alternative medicine

When deciding to use folk remedies, it must be remembered that oncology is not treated in this way. None of the known drugs can cure mutated cells, stop their reproduction and growth.

It is acceptable to use alternative medicine to alleviate the signs of the disease, such as weakness, dizziness, and other symptoms - strictly in consultation with your doctor. It is not recommended to take vegetable or animal raw materials on your own due to possible side effects.

Effects

In the presence of Kaposi's sarcoma, the prognosis depends on the individual characteristics of the organism and the type of the disease. As a rule, the slower the angiosarcoma progresses, the more effective medical manipulations and the longer lifespan.

But often the illness develops rapidly, which entails a quick adverse outcome - especially if they do not carry out proper treatment.

Thus, it can be noted that the disease is distinguished by its symptoms depending on the dominance of a certain degree into which it is divided. Treatment of pathology, regardless of the degree of manifestation will be carried out exclusively in a medical institution, and folk remedies can be taken only to alleviate the manifestation of painful symptoms.

At the same time, it must be remembered that any treatment, whether it is conservative or popular, must necessarily be agreed with the attending physician, and self-treatment is strictly prohibited!

Content

The prevalence of this disease is generally small, but Kaposi’s sarcoma is the first among malignant neoplasms affecting HIV patients, reaching 40–60%.

Herpes simplex virus type 8 (HHV-8, HHV-8, KSHV) is found in almost 100% of patients with Kaposi's sarcoma [1].

The risk groups for the development of Kaposi's sarcoma include:

  • HIV-infected men
  • elderly men of Mediterranean origin,
  • faces from equatorial africa
  • persons with transplanted organs (recipients).

Usually, the tumor has a purple color, but the color may have different shades: red, purple or brown. The tumor may be flat or slightly rise above the skin, is a painless spots or nodules. Almost always located on the skin, at least - on the internal organs. Kaposi's sarcoma is often combined with damage to the mucous membrane of the palate, lymph nodes. The course of the disease is slow. Detection of Kaposi's sarcoma in HIV infection provides the basis for the diagnosis of AIDS.

The histological structure of the tumor is characterized by a multitude of chaotically located thin-walled newly formed vessels and bundles of spindle-shaped cells. The tumor is infiltrated with lymphocytes and macrophages. The vascular nature of the tumor dramatically increases the risk of bleeding. However, it is not necessary to do a biopsy for suspected Kaposi's sarcoma. Kaposi's sarcoma is a special type of tumor, which often does not require not only verification of the diagnosis, but also its treatment. This may seem strange at first glance. This situation is due to the fact that an accurate diagnosis can be made without a biopsy, and the isolated treatment of Kaposi's sarcoma extremely rarely gives complete healing. Moreover, the treatment of Kaposi's sarcoma (due to its connection with the causative factors of the underlying disease) is usually palliative, that is, only aimed at reducing the symptoms of the disease.

  • Classic type
  • Endemic type
  • Epidemic type
  • Immune suppressive type

Classic type Edit

Distributed in Central Europe, Russia and Italy. The favorite localization of Kaposi’s sarcoma of the classical type is the feet, lateral surfaces of the lower leg, and the surfaces of the hands. Very rarely on mucous membranes and eyelids. Lesions are usually symmetrical, asymptomatic, but rarely itching and burning. The boundaries of the foci, as a rule, clear.

There are 3 clinical stages: 1. patchy 2. papular 3. tumor

Spotted. The earliest stage. The spots at this stage are reddish bluish or reddish brown in color from 1 mm to 5 mm, irregularly shaped. the surface is smooth.
Papular The elements in this stage are spherical or hemispherical in shape, of a tightly elastic consistency, from 2 mm to 1 cm in diameter. More often isolated. When merged, they form a flattened or hemispherical plaque. The surface of the plaques is smooth or rough (like orange peels).
Tumor. At this stage, the formation of single or multiple nodes. With a diameter of 1-5 cm, red-bluish or bluish-brown color. Soft or tight elastic consistency, merging and ulcerating.

The causative agent of Kaposi's sarcoma

It is assumed that the cause of the development of Kaposi's sarcoma is the herpes simplex virus type 8 (HHV8) in combination with a significant decrease in the human immune system, which is found in HIV infection in the AIDS stage. This virus is found in every third bisexual or gay, in heterosexuals - in 5% of cases. Constantly HHV-8 is detected in tissues affected by Kaposi's sarcoma. In neighboring areas, viruses are not detected.

Fig. 4. In the photo, herpes virus type 8 (HHV8).

Causes and spread

The true sources of disease formation are unknown. Experts suggest that the problem is formed when a patient is infected with a herpes virus type 8 - a poorly understood variant.

The appearance of Kaposi's sarcoma is accompanied by other malignant pathologies:

  • Lymphosarcoma - a group of rapidly progressive diseases affecting the blood and lymph section,
  • multiple myeloma - plasma cell cancer,
  • fungal mycosis - damage of lymphoid tissues,
  • lymphogranulomatosis - hyperplastic changes of lymphoid tissue of cancer etiology,
  • leukemia - blood cancer.

The formation of abnormal deviations takes place with a significant decrease in the functionality of the autoimmune system. Separate subgroups of the population are more susceptible to the development of the disease:

  • male,
  • infected with HIV
  • after transplantation of internal organs - kidneys, etc.,
  • elderly men living in the Mediterranean, equatorial Africa.

The unanimous opinion of experts: the initial stages of the pathological process are represented by a reactive process, provoked by an infectious lesion. True sarcoma appears later - when the disease continues to progress.

Pathogenesis of the initial stage

Kaposi's sarcoma, its initial stage is characterized by a peculiar color shade of the formed tumor - purple, reddish, brown, purple.
The neoplasm may be flat, slightly rise above the skin, be painless nodules or spots.

Manifestations are located on the dermis, occasionally affect the surface of the internal organs. The abnormal process is combined with damaged lymph nodes, mucous membranes of the upper palate. The progress of the disease is slow.

The tumor is the primary, early sign of the transition of HIV infection to AIDS. The histological structure of the formation is represented by multiple, chaotically located thin-walled blood vessels, bundles of spindle-shaped cellular structures. A characteristic difference is the infiltration of tumors by macrophages, lymphocytes.

The presence of blood lines increases the risk of spontaneous bleeding. In the process of diagnosis does not require a biopsy - the problem is a special type of tumor-like changes. The initial diagnosis accurately determines the variant of the pathology that has arisen.

Kaposi's sarcoma has pronounced symptoms - the primary formation of pathology begins with bluish-purple spots, gradually infiltrating into rounded discs or nodules. Other options for development - there are cutaneous papules similar to rashes of lichen planus.

The gradual progression of the nodes ends with the growth, the size of a walnut, hazelnut. Formations are characterized by:

  • dense, elastic consistency,
  • peeling of the surface layers of the epidermis,
  • creating a vascular network, tumors are formed from a group of small vessels.

Nodules provoke pain, aggravated by pressure. According to the indicated signs, it is impossible to catch the disease - the formations spontaneously dissolve, are reborn by scar tissue - depressed hyperpigmentation. What does it look like? It seems that the patient has a problem with normal pigmentation of the skin.

In women, pathology occurs 8 times less.

Localization

The classic variation of the disease is common among males of older age. The most common pathology is formed on the leg - foot, lateral parts of the leg, hands, and upper limbs.

Less commonly recorded on the face - the area of ​​the nose, cheeks, eyelids. The foci have a symmetrical shape, clearly delineated. The disease is asymptomatic, can provoke obsessive itching, burning. It is able to provoke the formation of pathological abnormalities of mucous integuments - in the oral cavity, forming tumors of the tongue and gums.

Damage to the skin and mucous membranes is recorded in 95% of cases - the disease is manifested by purple, black, brown spots, accompanied by severe puffiness. Affecting the oral cavity occurs in 30% of patients.

Epidemic

The formation of a form of the disease indicates infection with HIV infection. Pathological anomaly is characterized by:

  • education in the period up to 40 years
  • unusual, bright color formations,
  • mandatory involvement in the disease of the mucous membranes,
  • non-standard location of the problem - the tip of the nose, oral cavity (area of ​​the hard palate), upper limbs.

The problem is characterized by rapid, malignant progression, with the involvement of the internal organs and lymph nodes in the process.

Immunosuppressive

The disease is formed when uncontrolled use of the same name drugs - after the transplantation of a donor kidney. The disease is characterized by:

  • minimal symptomatic manifestations
  • stable chronic, benign course,
  • involvement in the problem of internal organs, lymph nodes as an exception.

The abolition of the drug leads to a gradual regression of the anomalous process.

How to treat

Kaposi's sarcoma requires adequate treatment. Therapy is based on increasing the efficiency of the autoimmune system of the body. An isolated course of the disease rarely gives the possibility of complete healing.

The physician prescribes a palliative course aimed at suppressing the main clinical signs of the disease.

Local treatment

Treatment of Kaposi's sarcoma includes a number of auxiliary methods:

  • cryotherapy
  • injection of chemotherapeutic drugs,
  • the introduction of interferon in the tumor,
  • imposition of applications and ointments on the problem areas of the dermis.

Indications for local irradiation - the existing large foci of damaged skin, the need to achieve a cosmetic effect.

Systemic therapy

Treatment with a systemic method of Kaposi's sarcoma is performed by a surgical procedure - the excision of problem lesions, followed by the use of radiation therapy. The classical form of pathology is cured in 40% of cases.

Generalized forms, especially in those infected with HIV, require the use of:

Highly Active Antiretroviral Therapy:

  • total duration of more than one year
  • suppression of the negative effects of viruses,
  • increase the efficiency of the autoimmune system.

Certain types of these drugs can suppress the vital activity of a herpes infection that provokes the formation of Kaposi's sarcoma.

Chemotherapy is carried out by Prospidin, Vincristine, Rosevin, Taxol, Bleomycin, etc. In parallel with this treatment, patients are recommended hormone therapy - against the background of pronounced side effects in the blood-forming organs.

Interferon treatment - medications are used as immunomodulators. Therapy is carried out by long courses, with periodic replacement of drugs.

The disease is well shown on individual photographs. Photo of Kaposi's sarcoma:

Further life after the discovery of a pathological deviation completely depends on the severity of the course of the disease, the stage, and the condition of the patient. If the abnormal deviation spreads with unreal speed, then the patient's life span may be reduced - from a couple of months to several years.

The subacute phase of the disease gives hope for the extension of life within the next years. The chronic variant of the pathology with a slow course is characterized by lengthening - up to 5-1o years.

Kaposi's sarcoma requires timely detection and constant supervision by medical professionals. In due course, therapy gives hope to almost 40% of patients, which in cancer tumors is considered a positive prognosis.

The exact fulfillment of the doctors' instructions will help hopeless patients to adequately live the time allotted to them. Medicines suppress negative symptomatic manifestations, relieve painful sensations.

Causes and risk factors for Kaposi's sarcoma

It has now been proven that Kaposi's sarcoma arises as a result of the action of the herpes virus type 8, which can be transmitted through blood, sexually or with saliva. However, for the occurrence of the pathological process requires a significant reduction in immunity.

Under the microscope, Kaposi's sarcoma looks like a large number of newly formed vessels and various cells.

The following populations are most susceptible to the disease:

  • HIV-infected patients
  • patients with immunodeficiency states (for example, during chemotherapy, taking immunosuppressants),
  • elderly people or children
  • представители некоторых национальностей (например, жители Африки, а также люди средиземноморского происхождения).

Когда обнаруживается саркома Капоши при ВИЧ-инфекции, это служит основанием к постановке диагноза СПИД. Also, this neoplasm often accompanies the following pathological processes: lymphogranulomatosis, lymphosarcoma, leukemia, and myeloma.

The disease is characterized by a slow, progressive course. Since the tumor is mainly composed of blood vessels, bleeding may occur if it is damaged. With a large amount of Kaposi's sarcoma, it can ulcerate and undergo necrosis. The presence of lesions on the limbs may be accompanied by hyperpigmentation of the skin, the development of elephantiasis.

Even after treatment, hyperpigmentation remains for a long time (possibly even for life) at the site of former tumors. Ivory also usually lasts a long time.

The appearance of Kaposi's sarcoma on the mucous membranes may be manifested by the following symptoms:

  • Difficulty eating and soreness in the appearance of tumors in the mouth.
  • Pain, diarrhea and bleeding with the defeat of the stomach and intestines.
  • Difficulty breathing, shortness of breath, cough with bloody sputum when the tumor is located on the respiratory tract mucosa.

Intestinal and pulmonary forms of the disease in the absence of therapy can lead to life-threatening conditions. If you suspect a diagnosis such as Kaposi's sarcoma, a photo of her most characteristic symptoms can be found on the Internet or any medical directory.

With a long course of the disease, a lesion of the lymph nodes, liver, bones and other organs can be observed.

Pathogenesis of the disease

A sharp decline in the work of the immune system leads to the activation of the herpes type 8 viruses, which cause a number of disorders in the patient's body. In the dermis, subcutaneous tissue and mucous membranes the proliferation of the endothelium of the inner wall of blood vessels is noted. The structure of the vessels themselves is destroyed, they become similar to the “sieve”. The erythrocytes begin to penetrate into the surrounding tissues, hemosiderin is deposited in their clusters. There is a marked proliferation of young fibroblasts, which have a spindle shape, some of them acquire signs of atypia. Detection of spindle-shaped cells is a histological marker of the tumor. In the lesions, macrophages and lymphocytes appear in large numbers. Lesions have the form of spots (roseol), plaques, nodules and tumor-like formations, which eventually suppurate and ulcerate.

The growth of AIDS-associated Kaposi's sarcoma is stimulated by:

  • glucocorticoid and immunosuppressive drugs (cyclosporine),
  • cytokines that produce HIV-infected mononuclear cells,
  • interleukin-6 (IL-6) and interleukin-1bet (IL-1b),
  • major fibroblastic growth factor,
  • vascular endothelial growth factor,
  • transforming growth factor beta,
  • tat protein HIV and others

Immunosuppression in AIDS patients is a major factor in the development of Kaposi's sarcoma.

Fig. 5. Kaposi's sarcoma in a patient with AIDS. Most often, the process is localized on the upper half of the body.

Clinical manifestations

Immunosuppression is one of the conditions for the development of Kaposi's sarcoma. With a relatively high number of CD4 lymphocytes in HIV-infected patients, the tumor develops slowly, and when the number of CD4 lymphocytes is below 200 in 1 μl, it develops quickly. With the development of pneumocystic pneumonia or with long-term use of corticosteroids, Kaposi's sarcoma in patients develops rapidly.

Features of Kaposi's sarcoma in AIDS patients

The main features of Kaposi's sarcoma in AIDS patients:

  • Young age - up to 35 - 40 years.
  • The development of the tumor process is noted in every third patient. These are mainly homosexual persons and patients with the presence of sexually transmitted diseases.
  • Sarcoma develops with other opportunistic infections (often with Pneumocystis pneumonia).
  • Localization of primary elements on the head, neck, upper half of the body and genitals.
  • The common nature of lesions (multifocal). Symmetry is not typical.
  • Hemorrhagic spots are often bright red, plaques and nodules are the main elements of the rash.
  • Rash often suppurate and ulcerate.
  • Rapid generalization of the tumor process with damage to the lymph nodes, lungs, gastrointestinal tract and oral mucosa.
  • Pronounced lymphostasis.
  • High mortality (life expectancy of 80% of patients does not exceed one and a half years).

The appearance of Kaposi's sarcoma in an HIV-infected patient indicates the transition of the disease to the AIDS stage.

Fig. 6. Kaposi's sarcoma of the small intestine. Picture taken with endoscopy.

Type of tumor

In HIV infection, lesions on the skin and mucous membranes have the form of spots (roseol), papules, plaques or nodules often purple color, less often have a purple or brown color. The boundaries of foci are clear. The elements of the tumor are painless, itching and burning of the patient is rarely disturbed. Over time, the rash becomes numerous, sometimes merging, forming tumor-like formations, which eventually begin to bleed, suppurate and ulcerate.

Fig. 7. Spots, papules, plaques and nodules are elements of Kaposi's sarcoma.

Kaposi's Sarcoma Forms

Skin lesions registered in 2/3 of cases. The process is characterized by rashes in the form of roseol, papules, plaques and nodules, usually purple or brown. The face, neck, upper half of the body and the anogenital region are the most frequent localization. The rash is painless, but with the appearance of inflammation, swelling and pain appear. Large tumor formations often suppurate and ulcerate.

The defeat of the mucous membranes. Rashes are most often common, localized to the nasal mucosa (22% of cases), mouth and throat. In the mouth, elements of the tumor appear on the soft and hard palate (5% of cases), cheeks, arms, tongue, larynx and pharynx. When the process is generalized, the lesions become sharply painful.

Lesion of internal organs noted in 75% of patients. The esophagus, intestines, spleen, liver, kidneys, lungs, heart and brain, where infiltrates occur, are affected. With the spread of the pathological process on the lung tissue in patients with shortness of breath. More than half of patients do not live up to 6 months.

Damage to the gastrointestinal tract (45% of cases) is often complicated by bleeding, which is fatal. In 61% of cases lymph nodes are affected.

Fig. 12. Kaposi's sarcoma of the conjunctiva of the eye (photo on the left) and sclera (photo on the right).

Stages of Kaposi's Sarcoma

Spotted. In the beginning there are spots (roseola). With AIDS, they have a purple or brown-red color, irregular rounded shape, 1 - 5 mm in diameter, with a smooth surface, painless, not accompanied by itching. Most often located on the face, nose, around the eyes, palms, genitals, on the skin of the upper half of the body.

Papular Elements of the rash begin to rise above the surface of the skin and turn into papules up to 1 cm in diameter. They have a hemispherical shape, dense, elastic, isolated. When merged, plaques with a rough surface ("orange peel") are formed.

Tumor. At the confluence of papules and plaques, tumor formations are formed. Large nodes are dense to the touch, bluish-red color, from 1 to 5 cm in diameter. Over time, become covered with ulcers and begin to bleed.

The use of highly active antiretroviral therapy slows the progression of Kaposi's sarcoma.

Fig. 13. Kaposi's sarcoma on the foot, tumor form.

Fig. 14. Kaposi's sarcoma of the leg and foot.

Fig. 15. Kaposi's sarcoma with HIV on the face: the initial form (photo left) and the common form (photo right).

Fig. 16. Kaposi's sarcoma with HIV infection on the face.

Fig. 17. Kaposi's sarcoma with HIV on the face: around the eyes (photo left) and on the forehead in the form of nodules (photo right).

Histological picture of the disease

Fig. 18. The photo on the left is an early form of the disease. The photo on the right shows the proliferation of spindle-shaped cells, isolated and clusters of red blood cells.

Fig. 19. In the photo, massive proliferation of spindle cells. Single erythrocytes and sites of accumulations of erythrocytes are visible.

Fig. 20. The photo shows extensive areas filled with blood that mimic a benign vascular tumor.

Fig. 21. The node in Kaposi's sarcoma is under the epidermis, which is not yet damaged (photo on the left). In the photo on the right, the superficial epidermis over the lesion with ulcerations.

Fig. 22. Kaposi's sarcoma with localization on the mucous membrane of the stomach (photo left), in the lymph node (photo right).

The diagnosis of Kaposi's sarcoma in HIV patients can be made accurately without a biopsy.

Prognosis of the disease

The prognostic criteria for Kaposi's sarcoma in AIDS patients are the clinical manifestations of the disease and the indicators of laboratory research methods.

Clinical manifestations that determine the prognosis of the disease:

  • The severity of the pathological process.
  • Involvement in the infectious process of internal organs.
  • The severity of intoxication syndrome.
  • The presence of opportunistic infections.

  • Hematocrit.
  • CD4 lymphocyte count.
  • The ratio of CD4 / CD8 lymphocytes.
  • Interferon alpha level.

To assess the prognosis of Kaposi's sarcoma helps the level of T-cells:

When the number of T-helpers is more than 300 in 1 μl in patients who have survived for more than 1 year, it is rated as good, from 300 to 100 - satisfactory, less than 100 - bad.

The ratio of CD4 / CD8 lymphocytes in patients who survived for more than 1 year, more than 0.5 is good, 0.5 - 0.2 is satisfactory, less than 0.2 is bad.

In patients with AIDS, the prognosis of Kaposi's sarcoma is unfavorable. 99 - 100% of patients with a common form of the disease die in the first 3 months of the disease.

Fig. 23. A common form of Kaposi's sarcoma in an AIDS patient. The prognosis is unfavorable.

Systemic chemotherapy

The use of this type of treatment is dictated by the aggressive course of sarcoma in the majority of AIDS patients, involvement of lymph nodes and internal organs in the pathological process. However, the presence of opportunistic diseases, anemia and immunodeficiency limit its use for many patients in need. Monotherapy is used for moderate manifestations of the clinic, combined chemotherapy is used for pronounced manifestations. There is no single approach to the use of chemotherapy for AIDS patients.

For the treatment of Kaposi's sarcoma in AIDS patients there are many chemotherapy drugs: Vinblastine (effect 26% effect) Etoposide (76%), Vincristine (61%), Bleomycin (77%), Vincristine + Vinblastine (45%), Bleomycin + Vinblastine (62%), Vinblastine + Methotrexate (77%), Doxorubicin + Bleomycin + Vinblastine (66 — 86%).

Broad-spectrum cytostatics

It has been proven to be very effective in AIDS patients with Kaposi’s sarcoma of broad-spectrum cytostatics - liposomal anthracyclines Daunksom and Doksil. Therapy Doksilom superior to the effectiveness of the "gold standard" treatment of Kaposi's sarcoma - Adriamycin + Bleomycin + Vincristine.

Active Antiretroviral Therapy

Active antiretroviral therapy with the use of 3 or more drugs has a prophylactic and therapeutic effect for Kaposi's sarcoma.

Fig. 24. Local treatment of Kaposi's sarcoma is applied in a localized form.

Treatment of Kaposi's sarcoma in AIDS patients is palliative. It is aimed only at reducing the severity of symptoms of the disease.

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