What Is Castleman Disease?

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About Castleman Disease?

Castleman disease is a rare disorder that involves an overgrowth (proliferation) of cells in your body’s disease-fighting network (lymphatic system). Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease can occur in a localized (unicentric) or widespread (multicentric) form.

Treatment and outlook vary, depending on the type of Castleman disease you have. The localized type can usually be successfully treated with surgery.

Sometimes associated with HIV infection, multicentric Castleman disease can be life-threatening. Multicentric Castleman disease is also associated with other cell-proliferation disorders, including cancer of the lymphatic system (lymphoma), Kaposi’s sarcoma and POEMS syndrome.

Castleman infection (Compact disc) depicts a gathering of three immunological issue that happen in people of any age and offer a comparable tiny lymph hub appearance. Dr. Benjamin Castleman first depicted the group of stars of lymph hub highlights saw in Album (“Disc like highlights”) in the 1950s. Roughly 6,600-7,700 patients are assessed to be determined to have Album consistently in the Assembled States. There is critical fluctuation in clinical highlights, medications, and survival over the three subtypes.

Castleman infection is an uncommon ailment of lymph hubs and comparative tissues. At times alluded to as Castleman’s ailment, it is really a gathering of purported lymphoproliferative scatters, which means issue that are joined by unusual development (or multiplication) of the lymphatic tissue. The lymphatic (or lymphoid) tissue comprises of various sorts of cells that are engaged with the insusceptible reaction, overwhelmingly white platelets known as lymphoctyes. Lymphatic tissue is found in lymph hubs, the tonsils, thymus, spleen, bone marrow, and gastrointestinal (GI) tract.

Strange development of lymphoid tissue additionally is normal for lymphoma, a kind of growth, yet Castleman ailment isn’t malignancy. Different names for Castleman illness are monster lymph hub hyperplasia and angiofollicular lymph hub hyperplasia (AFH). Despite the fact that Castleman malady isn’t thought to be tumor, one kind of this illness (multicentric Castleman infection, see beneath) carries on particularly like lymph hub growth (lymphoma), and can prompt genuine wellbeing dangers.

Symptoms

UCD is described by a solitary developed lymph hub or various extended lymph hubs in a solitary district of the body, for example, the chest, guts, or neck. By and large of UCD, people display no indications (asymptomatic). Once in a while, patients encounter indications because of the size and area of the development. For instance, a development may frame by a vein, bringing about a lump and conceivable check in the included vein. Infrequently, people with UCD may show an assortment of side effects including fever, weakness, intemperate sweating, weight reduction, skin rash, early pulverization of red platelets, prompting bizarrely low levels of coursing red platelets (hemolytic pallor), as well as anomalous hoisted measures of certain safe factors in the blood (hypergammaglobulinemia). These side effects are normally observed in MCD. These indications ordinarily vanish after surgical extraction of the UCD lymph hub.

Both HHV-8-related MCD and iMCD are described by numerous districts of amplified lymph hubs and long winded provocative manifestations, for example, fever, weight reduction, skin rash, decimation of red platelets, prompting bizarrely low levels of flowing red platelets (hemolytic iron deficiency), and additionally anomalous expanded measures of certain resistant factors in the blood (hypergammaglobulinemia). Numerous people with MCD may display an anomalous expansive liver and spleen (hepatosplenomegaly).

HHV-8– related MCD is most regularly analyzed in HIV-tainted or generally immunocompromised people. In this manner, HHV-8-related MCD patients may encounter extra side effects identified with their HIV disease or different conditions.

There are two basic types of Castleman disease:

  • Unicentric Castleman disease.This localized form of the disease affects only a single gland (lymph node) in your lymphatic system.
  • Multicentric Castleman disease.This type affects multiple lymph nodes and lymphatic tissues, and can severely weaken your immune system.

Multicentric Castleman disease can be further classified as:

  • Multicentric Castleman disease without POEMS syndrome
  • Multicentric Castleman disease with POEMS syndrome that involves areas of abnormal bone (osteosclerotic lesions)
  • Multicentric Castleman disease with POEMS syndrome without osteosclerotic lesions

Unicentric Castleman disease

Many people with unicentric Castleman disease don’t notice any signs or symptoms. The diseased lymph node is usually located in the chest, neck or abdomen. When signs and symptoms are present, they may include:

  • A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
  • An enlarged lump under the skin in the neck, groin or armpit
  • Unintended weight loss
  • Less commonly, fever, night sweats and weakness

Multicentric Castleman disease

Most people with multicentric Castleman disease experience:

  • Fever
  • Night sweats
  • Fatigue and weakness
  • Loss of appetite
  • Unintended weight loss
  • Enlarged lymph nodes, usually around the neck, collarbone, underarm and groin areas
  • Enlarged liver or spleen

Other, less common symptoms include:

  • Nerve damage in the hands and feet that leads to numbness (peripheral neuropathy)
  • Skin rash

Causes

It’s not clear why you get Castleman disease. Part of it seems to be connected to problems with the immune system — your body’s main defense against germs.

If you have HIV, the virus that causes AIDS, you may have a higher risk of getting the multicentric form of Castleman disease. Your immune system is weak, and you’re more likely to get infected with another virus called HHV8. Scientists aren’t sure why or how, but this virus seems to be linked in some way to the growth of too many cells in the lymph nodes.

It’s not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease. The exact cause of UCD and iMCD is not known. Viruses, genetic mutations acquired over the course of life, and inflammation have all been proposed as possible causes of UCD. Recent research suggests that acquired genetic mutations are the likely cause of UCD.

HHV-8 is the well-established cause of HHV-8-associated MCD, which accounts for approximately 50% of all cases of MCD. HHV-8-associated MCD often occurs in individuals infected with human immunodeficiency virus (HIV). The HIV weakens the immune system’s ability to control the HHV-8 infection. The HHV-8 virus causes MCD by making its own IL-6 and causing cells to proliferate.

Approximately 50% of MCD cases are negative for HHV-8 and the cause is unknown or “idiopathic.” Recently, four possible causes have been hypothesized: a virus, genetic mutation acquired over the course of life, an inherited genetic mutation, or autoimmunity. Some researchers speculate that increased production of interleukin-6 (IL-6) for one of the above causes may be involved in the development of iMCD. IL-6 is a substance normally produced by cells within the lymph nodes (plasma cells) and in healthy individuals serves to coordinate the immune response to infection. However, IL-6 is not elevated in all cases, and neutralizing IL-6 is not effective for the treatment of all cases.

The HHV-8 virus has also been linked to the development of Kaposi’s sarcoma, a cancerous tumor of the blood vessel walls that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in HIV-positive people who have Castleman disease, and in 40 to 50 percent of HIV-negative people with Castleman disease. The precise role of HHV-8 is unclear. But it appears to cause malfunctioning immune system cells to reproduce rapidly. The immune system cells produce a protein called interleukin-6 (IL-6) that contributes to the overgrowth of lymphatic cells.

Risk factors

Castleman disease can affect anyone. But the average age of people diagnosed with unicentric Castleman disease is 35. Most people with the multicentric form are in their 50s and 60s. The multicentric form is also slightly more common in men than in women.

The only known risk factor for Castleman disease appears to be having HIV/AIDS.

Complications

People with unicentric Castleman disease usually do well once the affected lymph node is removed. However, having Castleman disease may increase your risk of lymphoma.

Complications of multicentric Castleman disease can be life-threatening and may include:

  • Infection leading to the failure of multiple organs
  • Cancer, such as lymphoma or Kaposi’s sarcoma

The outlook for people with multicentric Castleman disease varies, depending on the nature of their disease. The presence of HIV/AIDS tends to worsen the outcome.

Research also indicates that people who have multicentric Castleman disease with POEMS syndrome that doesn’t involve bone lesions may have worse outcomes, while people who have multicentric Castleman disease with the bone lesion variant of POEMS syndrome tend to do better.

Diagnosis

Most UCD patients don’t encounter fundamental side effects. Normally, the developed lymph hub will be found unintentionally, amid look after another condition, or in light of the fact that it is blocking on close-by organs. UCD is analyzed by histopathological examination of the extracted lymph hub.

There are no official indicative criteria for HHV-8-related MCD. It is by and large analyzed when a patient has different districts of amplified lymph hubs, provocative manifestations, biopsy of the lymph hub illustrates “Castleman-like” highlights under the magnifying lens, and HHV-8 testing is sure. Trademark “Castleman-like” infinitesimal highlights may incorporate a group of stars of relapsed or hyperplastic germinal focuses, follicular dendritic cell unmistakable quality, hypervascularization, and polytypic plasmacytosis. A demonstrative model for iMCD was as of late settled. The criteria require significant criteria (trademark lymph hub includes on biopsy and various districts of extended lymph hubs), at least 2 of 11 minor criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Laboratory and clinical minor criteria include elevated C-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, polyclonal hypergammaglobulinemia, constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis.

The diagnosis of all forms of Castleman disease should be based upon a thorough clinical evaluation that includes a detailed patient history and a variety of specialized imaging techniques (e.g., computer-assisted tomography [CT scan], magnetic resonance imaging [MRI], and ultrasonography). During CT scanning, a computer and X-rays are used to create a film showing cross-sectional images of an organ’s tissue structure. MRI uses a magnetic field and radio waves to create cross-sectional images of the organ. In ultrasonography, reflected sound waves create an image of the organs in question. These tests can identify enlarged lymph nodes. Then, surgical (excisional) biopsy of the lymph node is needed. Finally, the clinician should integrate the clinical history, imaging results, and microscopic report to make the diagnosis.

Treatment

The treatment of multicentric Castleman disease (MCD) varies based on the severity of the condition and whether or not the patient has an HIV and/or human herpes virus type 8 (HHV-8) infection. Possible treatment options include:[1][2][3]

  • Immunotherapy can be used to block the action of the interleukin-6 (IL-6), a protein that is produced in excess by the immune system of people with MCD
  • Chemotherapy may be recommended to slow the growth of lymphatic cells
  • Corticosteroid medications can reduce inflammation
  • Anti-viral drugscan block the activity of HHV-8 or HIV (in people who are infected by these viruses)

Surgery is often used to get a tissue sample to diagnose Castleman disease (CD). A lymph node biopsy (described in Tests for Castleman Disease) is usually a minor procedure, and patients can often go home afterward.

Surgery also works well to treat localized (unicentric) CD. The type of surgery depends on where the disease is located. If the affected lymph node or nodes are in a place that is easy to get to, such as in the armpit, then surgery is usually straightforward. In many cases the person may even be able to go home the same day after the surgery.

When the enlarged lymph nodes are in a place that is hard to get to, like deep in the chest or abdomen, surgery is more complex and might require a stay in the hospital for a few days after the operation.

Another option is to have radiation or treatment with chemotherapy or another drug first. This can shrink the lymph nodes or tumors, which can make them easier to remove with surgery. Surgery might also be used to help treat symptoms rather than to try to cure the disease. For example, the spleen can be removed if it has grown large and is causing symptoms.

Potential side effects of surgery depend on several factors, including the extent of the operation and a person’s health before surgery. Most people will have at least some pain after the operation, but it usually can be controlled with medicines if needed. Other problems can include reactions to anesthesia, damage to nearby organs during the operation, bleeding, blood clots in the legs, and skin infections at the incision sites. Even though Castleman disease is not a cancer, surgery is often used in much the same way as it is for cancer. You can read more in Cancer Surgery.

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