Filed under: Boomer's Health
Castleman disease is a rare illness that affects your lymph nodes and other immune-cell structures of your body.
Also known as giant lymph node hyperplasia and angiofollicular lymph
node hyperplasia, Castleman disease is classified as a
lymphoproliferative disorder. That means it involves a proliferation, or
overgrowth, of lymphatic cells. This makes Castleman disease similar to
cancers of the lymphatic system (lymphomas), which also are
characterized by cell overgrowth. Castleman disease isn't considered a
Castleman disease can occur in a localized or a more widespread form.
Treatment and outlook depend on which type of Castleman disease you
There are two basic types of Castleman disease:
* Unicentric Castleman disease. This localized form of the disease affects only a single lymph node.
* Multicentric Castleman disease. This type affects multiple lymph
nodes and lymphatic tissues, and can severely weaken your immune system.
It sometimes accompanies HIV/AIDS.
The two different types of Castleman disease affect people very differently.
Unicentric Castleman disease
Many people with unicentric Castleman disease notice no symptoms at all.
Most often, the diseased lymph node is located in the chest or abdomen.
When there are signs and symptoms, they may include:
* A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
* Low-grade fever
* Weight loss
* Skin rash
* Excessive sweating
Multicentric Castleman disease
People with multicentric Castleman disease usually develop systemic signs and symptoms including:
* Night sweats
* Loss of appetite
* Nausea and vomiting
* Weight loss
* Weakness or fatigue from anemia
* Enlarged peripheral lymph nodes, usually around the neck, collarbone, underarm and groin areas
* Enlarged liver or spleen
* Nerve damage in the hands and feet that leads to numbness or weakness (peripheral neuropathy)
When to see a doctor
If you notice an enlarged lymph node on the side of your neck or in your
underarm, collarbone or groin area, talk to your doctor. Also, call
your doctor if you have other signs or symptoms, such as a feeling of
fullness in your chest or abdomen, fever, fatigue or unexplained weight
loss. These signs and symptoms are common to many different types of
illnesses. See your doctor to determine the underlying cause.
The exact cause of Castleman disease remains unknown. Infection by a
virus called the human herpes virus 8 (HHV8) is associated with
Castleman disease, and doctors suspect that it may play a causal role,
especially in multicentric Castleman disease. This virus has also been
linked to the development of Kaposi's sarcoma, a cancerous tumor of the
blood vessel walls, which is also common in people with multicentric
Castleman disease. People who are HIV-positive are more likely to have
both Castleman disease and Kaposi sarcoma.
Researchers aren't clear what the precise role of HHV8 may be, though it
appears that it may help malfunctioning immune system cells reproduce
rapidly. The immune system cells are called interleukin-6 (IL-6), and a
protein produced by these cells may contribute to the overgrowth of
The HHV virus is likely transmitted through saliva, but may also be
passed from person to person via sexual contact, blood transfusions and
Generally, unicentric Castleman disease isn't associated with HHV8.
There don't seem to be any consistent risk factors for Castleman
disease, except for having HIV/AIDS. The disease can affect anyone —
adults and children, men and women. The average age of people with
unicentric Castleman disease is around 30 or 40, whereas most people
with the multicentric form are in their 50s and 60s.
Unicentric Castleman disease
People with unicentric Castleman disease usually do well once the
affected lymph node is removed, although having Castleman disease may
increase your risk of lymphoma.
Multicentric Castleman disease
On the other hand, multicentric Castleman disease is much more serious
and often life-threatening. Death from multicentric disease usually
occurs due to a serious infection, failure of multiple organs, or
cancer, such as lymphoma or Kaposi sarcoma. The presence of HIV tends to
worsen the outcome.
Preparing for your appointment
You're likely to start by seeing your family doctor or a general
practitioner. You may then be referred to a doctor who specializes in
treating blood disorders (hematologist).
Because appointments can be brief, and there's often a lot of ground to
cover, it's a good idea to be well prepared for your appointment. Here's
some information to help you get ready for your appointment, and what
to expect from your doctor.
What you can do
* Write down any symptoms you're experiencing, including any that
may seem unrelated to the reason for which you scheduled the
* Write down key personal information, including any major stresses or recent life changes.
* Make a list of all medications, vitamins and supplements that you're taking.
* Ask a family member or friend to come with you to your
appointment, if possible. Sometimes it can be difficult to soak up all
the information provided to you during an appointment. Someone who
accompanies you may remember something that you missed or forgot.
* Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions
will help you make the most of your time. List your questions from most
important to least important in case time runs out. For Castleman
disease, some basic questions to ask your doctor include:
* What's the most likely cause of my symptoms?
* Are there other possible causes for my symptoms?
* What kinds of tests do I need? Do these tests require any special preparation?
* Can surgery cure me?
* What's my prognosis?
* What medications are available, and which do you recommend?
* What types of side effects can I expect from these drugs?
* I have other health conditions. How can I best manage them together?
* Is there a generic alternative to the medicine you're prescribing me?
* Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?
In addition to the questions that you've prepared to ask your doctor,
don't hesitate to ask questions during your appointment at any time that
you don't understand something.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to
answer them may reserve time to go over any points you want to spend
more time on. Your doctor may ask:
* Do you have any other health conditions, such as HIV/AIDS or Kaposi's sarcoma?
* When did you first begin experiencing symptoms?
* Have your symptoms been continuous, or occasional?
* How severe are your symptoms?
* Does anything seem to improve your symptoms?
* What, if anything, appears to worsen your symptoms?
Tests and diagnosis
A number of tests can help your doctor determine if you have Castleman
disease, although unicentric Castleman disease may be found
incidentally. People with unicentric Castleman disease often notice no
symptoms, and the diseased lymph node is found during screening or
treatment for other illnesses, such as during a CT scan or during
abdominal or chest surgery.
Tests your doctor may conduct to obtain a diagnosis of either unicentric or multicentric Castleman disease include:
* Physical examination. Your doctor may examine not only your
swollen lymph nodes but also your other lymph nodes to determine their
size and consistency.
* Blood and urine tests. Blood and urine tests may help your doctor
rule out other infections or diseases. They can also reveal anemia and
abnormalities in blood proteins that are sometimes characteristic of
* Imaging techniques. An X-ray, computerized tomography (CT) scan or
magnetic resonance imaging (MRI) scan of your chest, neck, abdomen and
pelvis may detect the presence and number of enlarged lymph nodes. These
tests can also determine whether organs, such as your liver or spleen,
are enlarged. Positron emission tomography (PET) scans also may be used
in diagnosing Castleman disease and later, to assess whether a treatment
* Lymph node biopsy. To differentiate Castleman disease from other
types of lymphatic tissue disorders, such as lymphoma, it's necessary to
take a sample of lymph node tissue for examination in the laboratory.
If the enlarged lymph node is close to the surface of your skin, the
biopsy can be done under local anesthesia. If it's in your chest or
abdomen, more extensive surgery may be necessary to access the lymph
node. By looking at the structure of the cells, a doctor who specializes
in diagnosing disease (pathologist) may be able to tell whether it's
lymphoma or Castleman disease.
Because Castleman disease is rare, you may need a second opinion.
Treatments and drugs
Treatment depends largely on the type of Castleman disease you have.
Treatment for unicentric Castleman disease is almost always with
surgery, while multicentric Castleman disease requires more systemic
Unicentric Castleman disease
Unicentric Castleman disease can be cured by surgically removing the
diseased lymph node. If the lymph node is in your chest or abdomen —
which is often the case with unicentric disease — major surgery may be
required to remove the node. This usually involves a hospital stay. If
the affected node is in a place that's easy to access, such as in your
underarm area or your neck, a simpler procedure may be performed that
doesn't require hospitalization.
If surgical removal isn't possible, such as if the lymph node is
difficult to get to, radiation therapy may be an effective way to
destroy the affected tissue.
Multicentric Castleman disease
Treating multicentric Castleman disease is generally more difficult.
Because the disease is rare, has varied nonspecific signs and symptoms,
and spontaneously goes into remission at times, doctors have found it
difficult to identify the best treatment. In addition, there are no
clinical trials that offer definitive evidence in favor of any therapy.
It's also not clear if treatment should differ based on HIV status.
Most therapies are palliative, which means their goal is to relieve
signs and symptoms rather than cure the illness. Surgery usually isn't
an option for multicentric disease because of the number of lymph nodes
involved, although sometimes removing an enlarged spleen may help ease
Therapies that are commonly used for multicentric disease, with varying
degrees of success, include the following. Doctors generally try as many
therapies as they can to provide the most relief.
* Corticosteroids. The basic function of corticosteroid therapy is
to control inflammation. With multicentric Castleman disease, doctors
usually prescribe steroids, such as prednisone, in combination with
other therapies. Long-term use of steroids may reduce your resistance to
infection, as well as increase your blood pressure, cause weight gain
and weaken your bones.
* Chemotherapy. Anti-cancer drugs (chemotherapy) can be useful
because they target rapidly reproducing cells, such as those associated
with Castleman disease. If chemotherapy is stopped, though, symptoms
tend to return and further treatment is needed to maintain symptom
relief. Still, chemotherapy has helped the disease go into remission for
some people. Usually, doctors use a combination of anti-cancer drugs.
Side effects may include hair loss, nausea, vomiting, loss of appetite
and reduced resistance to infection.
* Antiviral drugs. Antiviral medications, such as ganciclovir, are
known to inhibit the activity of the virus HHV8. The success of this
therapy has been mixed — effective for some people, but not for others.
Doctors still have many questions about antiviral therapy for Castleman
disease, such as which antiviral is best and when the best time to
administer it is.
* Monoclonal antibodies. These man-made antibodies are designed to
zero in on specific cells in the body that aren't working right. One
such antibody that may be effective for multicentric Castleman disease
works by neutralizing or blocking the activity of IL-6, the immune-cell
protein suspected of playing a role in causing the disease. Another
monoclonal antibody that may help is rituximab (Rituxan), a drug that
targets a different type of cell associated with Castleman disease.
* Immune modulators. Doctors have tried other drugs that help to
regulate the immune system (immune modulators) in the hopes that these
drugs might have some effect. Interferon alpha has both immunoregulatory
and antiviral effects and has been used successfully in a few people
with Castleman disease, although symptoms usually return once you stop
taking the drug. Thalidomide, a powerful immunomodulatory drug, also has
helped a few people and may help decrease the production of IL-6.
Coping and support
Because Castleman disease is so rare, it can be difficult to discover
you have the illness. Dealing with multicentric disease, in particular,
can be challenging. Some people with Castleman disease may have to deal
with multiple diseases, including HIV infection, AIDS and Kaposi's
sarcoma. In the face of a serious illness, it often helps to:
* Learn all you can about Castleman disease. Learn more about how
the disease progresses, your prognosis and your treatment options,
including both experimental and standard treatments and their side
effects. The more you know, the more active you can be in your own care.
However, be aware as you read about Castleman that much of what is
known about the disease comes from small studies or case reports, so
this information may not uniformly apply to your situation.
* Be proactive. Although you may often feel tired and discouraged,
don't let others — including your family or your doctor — make important
decisions for you. Take an active role in your treatment.
* Maintain a strong support system. Strong relationships are crucial
in dealing with life-threatening illnesses. In addition to friends and
family, a counselor or a spiritual advisor also can be helpful. It's
unlikely that you'll be able to find a local support group for Castleman
disease, but check with your doctor to find out if he or she knows of
anyone else in your area with the disease who might want to connect. You
can likely find others with the disease to talk to online. The
International Castleman's Disease Organization has a forum on their Web
site for questions from people who've just been diagnosed.