Description

Castleman's disease is a group of rare diseases that involves the lymph nodes become larger, so-called enlarged lymph nodes, and a wide range of symptoms. The most common form of the disorder involves a single enlarged lymph nodes. This lymph node is usually in the chest or neck, but can occur in other areas of the body. This form of the disorder is called unicentric of Castleman's disease (UCD).

The Castleman's disease (MCD) is in a number of regions of enlarged lymph nodes, inflammatory symptoms and problems with the function of the organ. There are three types of MCD:

  • The HHV-8-associated MCD. This type is linked to human herpes virus type 8, called HHV-8, and the human immunodeficiency virus (HIV).
  • MCD idiopathic.The cause of this type is unknown. This is also called HHV-8-negative MCD. The most severe form of this type of MCD is known as iMCD-TAFRO. This condition gets its name from the symptoms that it causes.
  • POEMS-associated MCD. This type is linked to another condition called POEMS syndrome. POEMS syndrome is a blood disorder that damages the nerves and affects other parts of the body.

MCD idiopathic. The cause of this type is unknown. This is also called HHV-8-negative MCD.

The most severe form of this type of MCD is known as iMCD-TAFRO. This condition gets its name from the symptoms that it causes.

Sometimes, people may have 2-3 enlargement of the lymph nodes and mild symptoms that do not meet the criteria for the diagnosis of MCD. These people may have another illness, or they may have recently described subtype of Castleman's disease is called oligocentric of Castleman's disease. This subtype is rare.

Treatment and expectations vary depending on the type of Castleman's disease. Unicentric of Castleman's disease, which is the type that it is only an enlargement of the lymph nodes, they can usually be successfully treated with surgery.

The best treatment for oligocentric of Castleman's disease, which consists of a pair of enlarged lymph nodes and has limited symptoms, it is not known, but is thought to be similar to the treatment of unicentric of Castleman's disease.

While not all people with ecm to respond to the first treatment, there are medications that work to treat HHV-8-associated MCD and MCD idiopathic.

Symptoms

Many people with unicentric of Castleman's disease do not notice any signs or symptoms. The enlargement of the lymph nodes may be found during a physical exam or a test image for a different problem.

Some people with unicentric of Castleman's disease may have signs and symptoms that are seen with more frequency in Castleman's disease. These may include:

Body symptoms

  • Fever.
  • Weight loss that happens without trying.
  • Fatigue.
  • Night sweats.
  • The swelling.
  • Enlargement of the liver or spleen.

The blood of the symptoms

  • Low count of red blood cells, also called anemia.
  • High or low platelet count.
  • Higher levels of creatinine that to happen, because the kidneys do not function correctly.
  • Higher levels of antibodies known as immunoglobulins.
  • Low levels of a blood protein called albumin.

The symptoms of the more severe form of the disease, idiopathic MCD called iMCD-TAFRO are:

  • Low platelet count, also called thrombocytopenia.
  • Swelling and fluid in the body, known as anasarca.
  • Fever or a higher level of C-reactive protein, a marker of inflammation.
  • Reticulin fibrosis, which is checked by taking a sample of bone marrow.
  • The swelling of the organ, also called organomegaly.

When to see a doctor

If you see an enlargement of the lymph nodes on the side of your neck or in the armpit, collarbone or the groin area, talk to your health professional. Also call your health care team if you have a long-lasting feeling of fullness in the chest or abdomen, fever, fatigue, or weight loss that can not be explained.

Causes

It is not clear what causes unicentric of Castleman's disease or idiopathic multicentric Castleman's disease (MCD). However, the HHV-8-positive MCD is known to occur in people who do not have the typical function of your immune system due to HIV or other causes.

Risk factors

Castleman disease can affect people of any age or sex. People are usually diagnosed with Castleman's disease during middle age, but can occur at any age, even during childhood.

There is No known risk factors for unicentric of Castleman's disease or idiopathic multicentric Castleman's disease. Infection with HIV or have a condition that decreases the well-functioning of the immune system increases the risk of HHV-8-positive multicentric Castleman's disease.

Complications

People with unicentric of Castleman's disease (UCD) usually do well once the affected lymph nodes are removed, and the life expectancy is usually not changed. But they are at higher risk of developing a rare autoimmune disease called pemphigus paraneoplastic. This condition can be life-threatening. Pemphigus paraneoplastic cause blisters in the mouth and on the skin that are often misdiagnosed. Although the risk of developing pemphigus paraneoplastic is low, which checks for this condition is important if you have UCD.

Multicentric Castleman idiopathic disease can worsen rapidly to involve life-threatening problems with the function of the organ. This requires critical care with a machine that helps you breathe, call a fan, and the treatments that help with organ function, such as dialysis and transfusions.

The HHV-8-positive Castleman's disease may involve life-threatening infections and organ failure. People with HIV/AIDS in general have worse outcomes.

Diagnosis

After reviewing your medical history and perform a detailed physical exam, your health care professional may recommend:

  • Blood and urine tests. These can help rule out other infections or diseases. These tests can also be found anemia and changes in the proteins in the blood that can be typical of Castleman's disease.
  • Imaging tests. These tests can be found enlarged lymph nodes or an enlarged liver or spleen. A ct scan of neck, chest, abdomen and pelvis can be used. A positron emission tomography scan, also known as PET, can be used for the diagnosis of Castleman's disease. The PET may also show if the treatment is working.
  • Lymph node biopsy. This test is essential to diagnose Castleman's disease and rule out the related disorders, such as lymphoma. In a biopsy, a sample of tissue from an enlarged lymph node is removed and looked at in a lab.

Treatment

The treatment depends on the type of Castleman's disease.

Unicentric disease Castleman

The surgery to remove the affected lymph nodes is the usual treatment for unicentric of Castleman's disease (UCD). If the lymph nodes in the chest or in the abdomen, more surgery may be needed.

The surgery to remove the enlarged lymph nodes are usually cure UCD. However, the UCD, sometimes coming from behind. If surgery is not possible, you may need medications that are normally used for Castleman's disease. If the medications do not work, radiation therapy may be an option.

More research into the treatment of oligocentric of Castleman's disease is necessary, but the treatment is generally similar to that of UCD.

It is likely that you need to follow-up examinations, including imaging and laboratory tests, to check that the disease has not been back.

The HHV-8-positive Castleman's disease

Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. Rituximab is very effective, but sometimes, the medications called antiviral drugs and chemotherapies are required. Antiviral drugs can block the activity of HHV-8 and HIV, and chemotherapy can be used to get rid of the excess cells of the immune system.

Idiopathic multicentric Castleman's disease

Siltuximab (Sylvant) is usually the first treatment for MCD idiopathic. In the united states, siltuximab is the only medication approved by the Food and Drug Administration (FDA) for the treatment of MCD idiopathic. The people who get better after taking siltuximab tend to have long-term success of the treatment. This drug blocks the action of a protein called interleukin-6. The bodies of the people who have MCD idiopathic produce too much of this protein.

People who are critically ill with MCD idiopathic often treated with medications called corticosteroids. You may also need chemotherapy. Corticosteroids such as prednisone may help to control the inflammation. Chemotherapy can be used to get rid of the immune cells that are causing problems.

When siltuximab is not working, other treatments, such as rituximab (Rituxan) and sirolimus (Rapamune) can be used.

Preparing for your appointment

You may be referred to a physician trained in the treatment of disorders of the blood. This type of doctor is called a hematologist.

What you can do

  • Write down any symptoms you have been experiencing, and for how long.
  • Write down your key medical information, including other health conditions.
  • Make a list of all the medicines, vitamins, and supplements you are taking, including the dosage.

Questions to ask your doctor

  • What is the most likely cause of my symptoms?
  • What kinds of tests do I need? Does not require special preparation?
  • What treatment do you recommend it? I need surgery?

In addition to the questions that you've prepared to ask your health care professional, do not hesitate to ask questions during your appointment.

What to expect from your doctor

Your health care team is likely to ask a series of questions. Be prepared to answer them you can let go the more points you want to spend more time. You may ask:

  • When was the first you have symptoms?
  • The symptoms been continuous or occasional?
  • How bad are the symptoms?
  • Nothing seems to improve the symptoms?
  • What, if anything, appears to worsen your symptoms?
  • You have other health problems, such as lymphoma, HIV/AIDS and kaposi sarcoma?
Symptoms and treatment of Castleman's disease