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Hhv-8-associated mcd

The meaning of «hhv-8-associated mcd»

Human herpesvirus 8 associated multicentric Castleman disease (HHV-8-associated MCD) is a subtype of Castleman disease (also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia), a group of rare lymphoproliferative disorders characterized by lymph node enlargement, characteristic features on microscopic analysis of enlarged lymph node tissue, and a range of symptoms and clinical findings.

People with human herpesvirus 8 associated multicentric Castelman disease (HHV-8-associated MCD) have enlarged lymph nodes in multiple regions and often have flu-like symptoms, abnormal findings on blood tests, and dysfunction of vital organs, such as the liver, kidneys, and bone marrow.

HHV-8-associated MCD is known to be caused by uncontrolled infection with the human herpesvirus 8 virus (HHV-8) and is most frequently diagnosed in patients with human immunodeficiency virus (HIV). HHV-8-associated MCD is treated with a variety of medications, including immunosuppressants, chemotherapy, and antivirals.

Castleman disease is named after Dr. Benjamin Castleman, who first described the disease in 1956. The Castleman Disease Collaborative Network is the largest organization focused on the disease and is involved in research, awareness, and patient support.

People with HHV-8-associated MCD may experience enlarged lymph nodes in multiple lymph node regions; systemic symptoms, such as fever, night sweats, unintended weight loss, and fatigue; rashes such as cherry hemangiomas or Kaposi sarcoma; enlargement of the liver and/or spleen; and extravascular fluid accumulation in the extremities (edema), abdomen (ascites), or lining of the lungs (pleural effusion).[1]

HHV-8-associated MCD is most commonly diagnosed in patients with human immunodeficiency virus (HIV), but can be seen in patients without HIV as well.[2]

Patients with HHV-8-associated MCD are often found to have Kaposi sarcoma,[1] a cancer caused by the HHV-8 virus and most commonly seen in patients with HIV. Patients with HHV-8-associated MCD have been observed to be at greater risk of developing lymphoma.[3]

HHV-8-associated MCD is known to be caused by infection with human herpesvirus-8.[4] The HHV-8 virus is commonly found in healthy individuals showing no signs of disease,[5] but it is also known to cause diseases such as Kaposi sarcoma and HHV-8-associated MCD. Diseases caused by HHV-8 are seen most frequently in the setting of immune dysfunction related to factors such as HIV infection or use of immunosuppressant medications; however, HHV-8-associated diseases, including HHV-8-associated MCD, have been reported in otherwise healthy individuals.[2]

In HHV-8-associated MCD the HHV-8 virus infects B cells and plasmablasts in lymph nodes and causes infected cells to release proinflammatory cytokines, signaling molecules that increase the activity of immune cells. In particular, HHV-8 infection of immune cells leads to increased levels of Interleukin-6 (IL-6), a cytokine known to play a role in other forms of Castleman disease.[citation needed]

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