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Kaposi sarcoma

  • Overview


    Kaposi sarcoma, a cancer originating from blood and lymph vessel linings, leads to the development of cell growths known as lesions on the skin. These distinctive lesions often emerge on the face, arms, legs, or even the genital area and mouth. Their colors range from pink and red to purple or brown.

    In severe cases, these lesions can manifest within the digestive tract and lungs. The underlying cause of Kaposi sarcoma is infection with human herpes virus 8 (HHV-8), also referred to as HHV-8. While the immune system keeps this infection in check for most healthy individuals, those with weakened immune systems may experience HHV-8 leading to Kaposi sarcoma.

    Kaposi sarcoma presents in four primary forms:

    1. AIDS-Related or Epidemic Kaposi Sarcoma: Found in individuals with human immunodeficiency virus (HIV), this form is closely linked to HIV/AIDS.
    2. Transplant-Associated or Iatrogenic Kaposi Sarcoma: Emerging after organ transplants, this type occurs in those who require immune-suppressing medications post-transplant.
    3. Classic Kaposi Sarcoma: Commonly affecting elderly men of Eastern European, Mediterranean, or Middle Eastern descent, this variant typically progresses slowly and may cause localized swelling, often in the legs.
    4. Endemic Kaposi Sarcoma: Seen predominantly in young individuals in Africa, this form can exhibit gradual skin growth or rapid internal spread.

    Understanding the distinct types and their causes is crucial for effective diagnosis, management, and treatment of Kaposi sarcoma. Regular medical assessments and tailored approaches are vital, especially for individuals at higher risk due to immune system vulnerabilities.

  • Symptoms


    Kaposi sarcoma can present in different forms based on various factors, including the underlying health status of the individual. Four primary types are recognized:

    1. AIDS-Related or Epidemic Kaposi Sarcoma: This type often occurs in individuals with HIV, the virus that causes AIDS. In this context, Kaposi sarcoma can indicate a weakened immune system. Lesions may appear on the skin, in the mouth, or other internal organs, leading to potential complications like difficulty swallowing, breathing problems, or gastrointestinal disturbances.
    2. Transplant-Associated or Iatrogenic Kaposi Sarcoma: People who have received organ transplants and are on immune-suppressing medications to prevent organ rejection may develop this type. Lesions can occur on the skin and internal organs, potentially impacting organ function and overall health.
    3. Classic Kaposi Sarcoma: Often seen in older individuals of Eastern European, Mediterranean, or Middle Eastern descent, this type tends to progress slowly. Skin lesions are a common feature, particularly on the lower extremities, and may lead to localized swelling.
    4. Endemic Kaposi Sarcoma: Primarily affecting people in certain regions of Africa, this form can vary widely in its presentation. Skin lesions are a hallmark, and their appearance and progression can be diverse, affecting both the skin and internal organs.

    In addition to the presence of lesions, other general symptoms may accompany Kaposi sarcoma, especially as the disease progresses. Fatigue is a common complaint, which can result from the body’s response to the cancer and the underlying immune system changes. Unexplained weight loss may occur due to the body’s increased energy expenditure and altered metabolic processes.

    Individuals with Kaposi sarcoma may also experience pain or discomfort, particularly if the lesions affect sensitive areas or internal organs. Respiratory symptoms can arise if the disease involves the lungs, leading to cough, shortness of breath, and chest pain. If the lesions affect the digestive tract, symptoms might include difficulty swallowing, nausea, vomiting, or abdominal pain.

  • When to see a doctor


    Abnormal symptoms may be a warning sign of potential dangerous diseases. Please contact our team of doctors immediately for detailed advice and update the most accurate and appropriate health care method.

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  • Causes


    The key trigger for Kaposi sarcoma is human herpesvirus 8 (HHV-8), also known as Kaposi’s sarcoma-associated herpesvirus (KSHV). HHV-8 is a member of the herpesvirus family, and it is the central player in the pathogenesis of Kaposi sarcoma. This virus infects cells lining blood vessels and lymphatic vessels, causing these cells to become cancerous over time. HHV-8 is most commonly transmitted through saliva, sexual contact, and organ transplantation.

    In people with healthy immune systems, HHV-8 infection usually remains latent, causing no noticeable symptoms. However, in individuals with compromised immune systems, such as those with HIV/AIDS or those undergoing organ transplantation and taking immunosuppressive drugs, HHV-8 can become reactivated and lead to the development of Kaposi sarcoma.

    One of the most significant risk factors for Kaposi sarcoma is HIV infection. People with HIV have weakened immune systems, which allows HHV-8 to become reactivated and replicate uncontrollably. The resulting uncontrolled viral replication leads to the formation of Kaposi sarcoma lesions on the skin and other organs. These lesions are a manifestation of the immune system’s struggle to control the virus and its oncogenic potential.

    Organ transplant recipients are another group at risk for Kaposi sarcoma. The immune-suppressing medications that transplant recipients must take to prevent organ rejection create an environment in which HHV-8 can reactivate and induce the formation of cancerous lesions. This is known as transplant-associated or iatrogenic Kaposi sarcoma.

    Genetic factors also play a role in Kaposi sarcoma. Some people may be more genetically predisposed to HHV-8 infection and its associated complications. Nevoid basal cell carcinoma syndrome, also called Gorlin-Goltz syndrome, is an inherited condition characterized by multiple basal cell skin cancers, along with various other symptoms. People with this syndrome have a higher risk of developing Kaposi sarcoma due to their genetic susceptibility to HHV-8.

    The interaction between HHV-8 infection, compromised immune systems, and genetic predisposition creates a complex web of causation that leads to the development of Kaposi sarcoma. The virus itself is the initiating factor, infecting blood vessel cells and inciting a series of events that result in the uncontrolled growth of cancerous lesions. Immune suppression, whether due to HIV infection or organ transplantation, allows the virus to flourish and evade the immune system’s control mechanisms. Genetic factors then contribute to an individual’s susceptibility to HHV-8 infection and the subsequent development of Kaposi sarcoma.

    While the precise mechanisms that lead from HHV-8 infection to Kaposi sarcoma are still under investigation, the intricate interplay between viral infection, immune suppression, and genetic factors highlights the multifaceted nature of this disease’s causation. Advances in our understanding of these causes provide crucial insights for the development of targeted therapies and interventions to prevent and treat Kaposi sarcoma.

  • Risk factors


    While its occurrence is less common compared to other types of cancer, certain risk factors have been identified that can increase an individual’s susceptibility to developing Kaposi sarcoma. Understanding these risk factors is essential for early detection, prevention, and effective management of the disease.

    1. Human Herpesvirus 8 (HHV-8) Infection: The primary risk factor for Kaposi sarcoma is infection with human herpesvirus 8, also known as Kaposi’s sarcoma-associated herpesvirus (KSHV). HHV-8 is a sexually transmitted virus that is transmitted through various forms of close contact, including sexual activity, saliva exchange, and blood transfusion. In regions where HHV-8 is prevalent, such as sub-Saharan Africa and certain Mediterranean countries, the risk of Kaposi sarcoma is higher due to increased exposure to the virus.
    2. Immunosuppression: Individuals with weakened immune systems are at a significantly higher risk of developing Kaposi sarcoma. This includes people living with human immunodeficiency virus (HIV) infection, as well as those who have undergone organ transplantation and require immunosuppressive medications to prevent organ rejection. The compromised immune response allows HHV-8 to reactivate and proliferate, leading to the formation of cancerous lesions.
    3. HIV/AIDS: Kaposi sarcoma is strongly associated with HIV/AIDS, particularly in individuals with advanced HIV infection. The weakened immune system in people with AIDS provides an ideal environment for HHV-8 to cause uncontrolled tumor growth. Kaposi sarcoma is often considered an AIDS-defining illness, and its presence can indicate the progression of HIV infection to AIDS.
    4. Gender and Age: Kaposi sarcoma has a higher prevalence in males than in females. Additionally, the disease is more commonly observed in older adults, particularly those over the age of 50. Classic Kaposi sarcoma, a subtype of the disease, is more frequently diagnosed in older individuals of Eastern European, Mediterranean, or Middle Eastern descent.
    5. Geographical Location: The prevalence of Kaposi sarcoma varies based on geographic location. Regions with a higher incidence of HHV-8 infection, such as sub-Saharan Africa and certain Mediterranean countries, also have a higher prevalence of Kaposi sarcoma. In these areas, the disease is more commonly associated with endemic forms of HHV-8 infection.
    6. Genetic Factors: While the genetic predisposition to Kaposi sarcoma is not fully understood, certain genetic factors may play a role in an individual’s susceptibility to HHV-8 infection and subsequent development of the disease. Some individuals may be inherently more susceptible to viral infection and tumor development due to their genetic makeup.
    7. History of Transplantation: Organ transplant recipients are at an increased risk of Kaposi sarcoma due to the use of immunosuppressive medications to prevent organ rejection. These medications weaken the immune response and allow HHV-8 to reactivate, leading to the development of cancerous lesions.
    8. Personal or Family History: People with a personal or family history of Kaposi sarcoma or other conditions associated with HHV-8 infection may be at an elevated risk. Additionally, individuals with a history of other cancers or conditions that compromise the immune system may have an increased susceptibility to Kaposi sarcoma.
  • Prevention


    Preventing Kaposi sarcoma primarily revolves around reducing the risk of human herpesvirus 8 (HHV-8) infection, as this virus is a key contributor to the development of the disease. While complete prevention may not be possible, several strategies can help mitigate the risk of HHV-8 infection and subsequently lower the chances of developing Kaposi sarcoma.

    1. Safe Sexual Practices: Practicing safe sex, including consistent and correct condom use, can reduce the risk of HHV-8 transmission through sexual activity. Limiting the number of sexual partners and avoiding risky sexual behaviors can further decrease the likelihood of HHV-8 infection.
    2. Blood Safety: Ensuring the safety of blood and blood products is crucial. Screening blood donations for HHV-8 can help prevent the transmission of the virus through blood transfusions.
    3. Organ Transplant Guidelines: For individuals receiving organ transplants, following recommended guidelines for immunosuppressive medications and regular medical check-ups is essential. Close monitoring can help detect HHV-8 infection early and prevent its progression to Kaposi sarcoma.
    4. Healthy Lifestyle: Maintaining a strong and healthy immune system is important for preventing Kaposi sarcoma. This includes adopting a balanced diet rich in fruits, vegetables, and whole grains, staying physically active, getting adequate sleep, and managing stress.
    5. Screening and Early Detection: Individuals at higher risk of Kaposi sarcoma, such as those with HIV/AIDS, should undergo regular medical check-ups and screenings. Early detection of HHV-8 infection and any suspicious skin lesions can lead to timely intervention and treatment.
    6. Vaccines: Although there is currently no specific vaccine for HHV-8, vaccines that prevent other infections, such as hepatitis B and human papillomavirus (HPV), can indirectly contribute to reducing the risk of Kaposi sarcoma by maintaining overall health and preventing other conditions that weaken the immune system.
    7. Education and Awareness: Raising awareness about the modes of HHV-8 transmission and risk factors associated with Kaposi sarcoma is crucial. Education empowers individuals to make informed decisions about their sexual health, blood safety, and overall well-being.
  • *Please note that the information provided in the article is for reference purposes only. It is essential to consult a doctor before applying any of the suggestions mentioned.

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Medical info from Mayo Clinic, for reference only. Visit Hoan My for better advice.

Last updated on: 06/08/2023