Castleman disease

From WikiMD

Summary

(KA-sel-man dih-ZEEZ)A rare disorder in which benign (not cancer) growths form in lymph node tissue. There are two main ways that Castleman disease occurs: localized (unicentric) and multicentric. Unicentric Castleman disease affects only one group of lymph nodes in one part of the body, usually in the chest or abdomen. It may not cause symptoms. Multicentric Castleman disease affects many groups of lymph nodes and lymphoid tissue all through the body. It can weaken the immune system and cause problems such as infection, fever, weight loss, fatigue, night sweats, nerve damage, and anemia. People with Castleman disease have an increased risk of lymphoma. Also called angiofollicular lymph node hyperplasia and giant lymph node hyperplasia.

Castleman disease
Castleman disease

Signs and symptoms

The signs and symptoms of MCD are often nonspecific, and are mild in some people but life-threatening in others. Symptoms may include enlarged lymph nodes in multiple regions, fever, weight loss, nausea, rash, and/or an enlarged large liver and spleen. The disease is diagnosed based on the symptoms present, laboratory test results, imaging studies, and results of a biopsy of the lymph nodes which shows specific features when studied under the microscope.[1] In some cases, MCD is caused by human herpesvirus-8 (HHV-8) and is referred to as HHV-8-associated MCD. In other cases, the cause is not known and it is referred to as HHV-8 negative MCD, or idiopathic MCD (iMCD). Treatment of MCD is challenging, and treatment options and outcomes depend on the type and severity in each person.

Castelman disease
Castelman disease

Symptoms

The signs and symptoms of multicentric Castleman disease (MCD) are often nonspecific and blamed on other, more common conditions. They can vary but may include:

  • Fever
  • Enlarged lymph nodes
  • Night sweats
  • Loss of appetite and weight loss
  • Weakness and fatigue
  • Shortness of breath
  • Nausea and vomiting
  • Enlarged liver or spleen
  • Peripheral neuropathy
  • Skin abnormalities such as rashes and/or pemphigus

Less commonly (<10% of cases), people affected by MCD will have no signs or symptoms of the condition.

Other conditions associated with MCD include amyloidosis, POEMS syndrome, autoimmune disease, hemolytic anemia, and immune thrombocytopenic purpura (ITP).

Cause

In about half of people with multicentric Castleman disease (MCD), it is caused by human herpesvirus-8 (HHV-8), in which case it is called HHV-8-associated MCD. This type of MCD usually occurs in people with human immunodeficiency virus (HIV) or a weakened immune system due to other reasons, because the weakened immune system is not able to manage a HHV-8 infection. It is thought that the infection multiplies in the lymph nodes, leading to the release of high levels of inflammatory chemicals (particularly cytokines such as interleukin-6), causing the specific symptoms of MCD.

In the remaining half of cases of MCD, the cause is not known, in which case it is called HHV-8 negative MCD, or idiopathic MCD (iMCD). Possible factors that have been speculated to play a role in causing iMCD include being infected with a virus other than HHV-8, acquiring a genetic mutation that signals the release of substances that lead to the disease, inheriting a genetic mutation that predisposes a person to developing the disease, and autoimmunity (when the body's immune system mistakenly attacks healthy tissues). People with iMCD often also have high levels of cytokines, but the cause of their increased production in iMCD is not yet known.

Inheritance

To our knowledge, there is no evidence that multicentric Castleman disease is an inherited disease.

Diagnosis

The signs and symptoms of multicentric Castleman disease (MCD) are often nonspecific and blamed on other, more common conditions. However, if MCD is suspected, the following tests may be recommended to help establish the diagnosis and rule out other conditions that cause similar features:

Blood tests can be ordered to evaluate the levels of Interleukin-6 (IL-6) and other substances in the body, which can be elevated in people with MCD. They can also be helpful in ruling out other autoimmune conditions and infections that are associated with similar signs and symptoms Imaging studies (such as a CT scan, PET scan, MRI scan, and/or ultrasound) can help identify enlarged lymph node(s) and other health problems A biopsy of affected tissue, often a lymph node, is usually recommended to confirm the diagnosis

Treatment

Treatment of multicentric Castleman disease (MCD) is challenging, and no single treatment works for all people with the disease.[7] Treatment options may depend on the type of MCD (HHV-8-associated or idiopathic) as well as the severity of symptoms.

HHV-8-associated MCD is typically initially treated with rituximab to fight against immune cells called B lymphocytes. This treatment is reportedly highly effective for HHV-8-associated MCD. Antiviral medications (particularly for those with HIV) and/or chemotherapy may also be recommended.

Idiopathic MCD (iMCD) is typically initially treated with siltuximab or tocilizumab (which aim to control activity of interleukin-6) with or without corticosteroids. Unfortunately, about half of people do not improve with these therapies. In those with only mild symptoms, rituximab may be an alternative option for initial treatment. In very severe cases, adjuvant combination chemotherapy is also recommended. Other types of therapies may be recommended if the above therapies are not effective.

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

siltuximab (Brand name: Sylvant) - Manufactured by Janssen Biotech, Inc. FDA-approved indication: Treatment of patients with multicentric Castleman's disease (MCD) who are human immunodeficiency virus (HIV) negative and human herpesvirus-8 (HHV-8) negative.

Prognosis

The long-term outlook (prognosis) for people with multicentric Castleman disease (MCD) varies. In some cases, the condition may persist for several months or years without progressing (worsening overtime). Other people affected by MCD experience episodes of severe symptoms that may improve spontaneously or in response to treatment, only to recur at a later date. People with MCD who are also HIV-positive may be at an elevated risk for a severe form that is rapidly progressive and can lead to death within weeks.

In general, the prognosis is worse in affected people who do not receive appropriate treatment and in those with HIV-associated MCD.

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NIH genetic and rare disease info

Castleman disease is a rare disease.

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