Summary about Disease
Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8), is a virus that can cause Kaposi sarcoma (KS), a type of cancer that develops from the cells lining blood and lymphatic vessels. KSHV can also cause other conditions such as primary effusion lymphoma (PEL) and multicentric Castleman disease (MCD). KSHV infection is often asymptomatic, but in individuals with weakened immune systems, such as those with HIV/AIDS or who are taking immunosuppressant drugs, it can lead to the development of these diseases.
Symptoms
Symptoms vary depending on the disease KSHV causes.
Kaposi Sarcoma (KS):
Painless, flat, or raised lesions on the skin, often reddish-purple or brown in color. These can appear anywhere, including the face, inside the mouth, and on the genitals.
Lesions can also develop in the lungs, gastrointestinal tract, and other internal organs.
Swelling in the legs and feet (lymphedema) may occur.
Primary Effusion Lymphoma (PEL):
Accumulation of fluid in body cavities such as the pleural space (around the lungs), peritoneal cavity (around the abdomen), and pericardial space (around the heart).
Shortness of breath due to fluid around the lungs.
Abdominal distension due to fluid in the abdomen.
Multicentric Castleman Disease (MCD):
Enlarged lymph nodes throughout the body.
Fever, night sweats, weight loss, fatigue.
Anemia (low red blood cell count).
Swelling in the abdomen (ascites).
Skin rashes.
Peripheral neuropathy (nerve damage).
Causes
KSHV infection is the primary cause of KS, PEL, and some forms of MCD. The virus is thought to spread through saliva, sexual contact, and possibly through blood transfusions or organ transplantation. People with weakened immune systems are at a higher risk of developing KSHV-related diseases.
Medicine Used
Treatment options depend on the specific KSHV-related disease and its severity.
Kaposi Sarcoma (KS):
Local therapy: Cryotherapy, excision, radiation therapy, topical medications (e.g., imiquimod).
Systemic therapy: Chemotherapy (e.g., liposomal doxorubicin, paclitaxel), antiviral medications (e.g., cidofovir, ganciclovir, foscarnet), immunomodulatory agents (e.g., interferon-alpha).
HAART (Highly Active Antiretroviral Therapy): For HIV-associated KS, HAART to control HIV can significantly improve KS.
Primary Effusion Lymphoma (PEL):
Chemotherapy (e.g., combination regimens containing etoposide, cyclophosphamide, doxorubicin, vincristine, and rituximab).
Antiviral medications (e.g., ganciclovir, cidofovir) may be used in combination with chemotherapy.
Multicentric Castleman Disease (MCD):
Anti-IL-6 therapy: Siltuximab (a monoclonal antibody against interleukin-6) and tocilizumab (an IL-6 receptor antagonist).
Chemotherapy (e.g., rituximab, cyclophosphamide).
Antiviral medications (e.g., ganciclovir, cidofovir) may be used if KSHV replication is significant.
Corticosteroids may be used to manage inflammation.
Is Communicable
Yes, KSHV is communicable. It is believed to spread through saliva (most common), sexual contact, and potentially through blood transfusions or organ transplantation.
Precautions
Practice safe sex to reduce the risk of transmission through sexual contact.
Avoid sharing items that may be contaminated with saliva, such as toothbrushes, razors, and drinking glasses.
Individuals with weakened immune systems should take extra precautions to avoid exposure to KSHV, such as avoiding contact with individuals who have active KSHV-related diseases.
If you have HIV, ensure proper control with antiretroviral therapy to strengthen your immune system.
Healthcare workers should follow standard infection control precautions when handling bodily fluids or tissues from individuals with KSHV-related diseases.
How long does an outbreak last?
KSHV infection is typically lifelong. Outbreaks of Kaposi sarcoma (KS) can vary in duration. They might involve the appearance of a few lesions that remain stable for months or years, or they can progress rapidly with new lesions appearing over weeks or months. The duration of an outbreak depends on the individual's immune status, the specific treatment used, and the underlying disease (e.g., HIV/AIDS). PEL and MCD are more continuous disease processes rather than episodic outbreaks.
How is it diagnosed?
Physical Examination: Examination of skin lesions or enlarged lymph nodes.
Biopsy: A tissue sample from a lesion or lymph node is examined under a microscope to identify KSHV-infected cells. Immunohistochemistry can be used to detect KSHV proteins.
Blood Tests: PCR (polymerase chain reaction) can detect KSHV DNA in blood samples. Antibody tests can detect antibodies against KSHV, indicating past or present infection.
Imaging Studies: Chest X-rays, CT scans, and MRI scans may be used to assess the extent of disease in internal organs.
Cytology: Fluid samples from pleural effusions or ascites can be examined for KSHV-infected cells in cases of PEL.
Timeline of Symptoms
The timeline of symptoms can vary greatly.
Initial Infection: Many individuals are asymptomatic after initial KSHV infection.
Latent Infection: The virus remains latent (dormant) in the body without causing symptoms.
Immunosuppression: When the immune system weakens (e.g., due to HIV/AIDS or immunosuppressant drugs), the virus can reactivate and cause disease.
Kaposi Sarcoma (KS): Skin lesions may develop gradually over weeks to months. The progression can be slow or rapid depending on the individual's immune status.
Primary Effusion Lymphoma (PEL): Accumulation of fluid in body cavities can occur over days to weeks, leading to symptoms such as shortness of breath and abdominal distension.
Multicentric Castleman Disease (MCD): Symptoms such as fever, night sweats, and weight loss may develop gradually over weeks to months.
Important Considerations
HIV/AIDS: KSHV-related diseases are more common and aggressive in individuals with HIV/AIDS. Effective HIV management with HAART is crucial to prevent and treat KSHV-related diseases.
Immunosuppression: Individuals taking immunosuppressant drugs after organ transplantation or for autoimmune diseases are also at increased risk.
Early Diagnosis: Early diagnosis and treatment of KSHV-related diseases can improve outcomes.
Monitoring: Regular monitoring for signs and symptoms of KSHV-related diseases is important, especially in individuals at high risk.
Supportive Care: Supportive care, such as pain management and nutritional support, can improve the quality of life for individuals with KSHV-related diseases.