- Castleman Disease Center
- Cancer 101 Pictures Slideshow
- Breast Cancer Slideshow
- Skin Cancer Slideshow
- Patient Comments: Castleman Disease - Experience
- Find a local Doctor in your town
- Castleman disease definition and facts
- What is Castleman disease?
- What are the signs and symptoms of Castleman disease?
- What causes Castleman disease?
- Is Castleman disease inherited (genetic)?
- What are the types of Castleman disease?
- What are the stages of Castleman disease?
- What is the prevalence of Castleman disease?
- Is there a test to diagnose Castleman disease?
- What is the treatment for Castleman disease?
- What medications treat Castleman disease?
- Is there surgery to treat Castleman disease?
- Which specialties of health-care professionals treat Castleman disease?
- What questions should I ask the doctor about Castleman disease?
- What is the life expectancy and prognosis for a person with Castleman disease?
Castleman disease definition and facts
- Castleman disease is a rare condition affecting lymph nodes, and actually is a group of related conditions.
- Castleman disease is not cancer, although some of the methods used to treat Castleman disease also are used to treat cancers.
- There are two main forms of Castleman disease, unicentric (localized) and multicentric (found in different sites throughout the body).
- Signs and symptoms of Castleman disease occur most often with the multicentric form and can include
- The diagnosis of Castleman disease involves examination of a tissue sample (biopsy) by a pathologist.
- Treatment for Castleman disease is complex, and can involve a number of different types and classes of medications such as chemotherapy drugs, immunotherapy drugs, immunomodulator drugs, anti-viral drugs, and corticosteroids.
- Surgery may be used to treat unicentric Castleman disease or to help relieve symptoms in the multicentric form.
- Radiation therapy is another form of treatment sometimes used to destroy the abnormal tissue in Castleman disease.
- Giant lymph node hyperplasia and angiofollicular lymph node hyperplasia (AFH) are other names for Castleman disease.
What is Castleman disease?
Castleman disease is a rare disease of lymph nodes and similar tissues. Sometimes referred to as Castleman's disease, it is actually a group of so-called lymphoproliferative disorders, meaning disorders that are accompanied by abnormal growth (or proliferation) of the lymphatic tissue. The lymphatic (or lymphoid) tissue consists of different types of cells that are involved in the immune response, predominantly white blood cells known as lymphoctyes. Lymphatic tissue is found in lymph nodes, the tonsils, thymus, spleen, bone marrow, and gastrointestinal (GI) tract.
Abnormal growth of lymphoid tissue also is characteristic of lymphoma, a type of cancer, but Castleman disease is not cancer. Other names for Castleman disease are giant lymph node hyperplasia and angiofollicular lymph node hyperplasia (AFH).
Even though Castleman disease is not considered to be cancer, one type of this disease (multicentric Castleman disease, see below) behaves very much like lymph node cancer (lymphoma), and can lead to serious health risks.
What are the signs and symptoms of Castleman disease?
There are no predictable signs and symptoms that suggest a person has Castleman disease. Some people with the condition do not show any signs or symptoms, while others will have a variety of symptoms.
Castleman disease has two forms, unicentric and multicentric.
Unicentric Castleman disease symptoms and signs
- Unicentric Castleman disease is localized and generally does not cause symptoms, although some affected people may have the symptoms associated with the multicentric form.
- People with unicentric Castleman disease have enlargement of lymph nodes in one area of the body.
- This form is much more common than multicentric Castleman disease, and is more likely to affect younger people.
Multicentric Castleman symptoms and signs
- Multicentric Castleman disease is more widespread, with abnormal enlargement of lymph nodes in locations throughout the body.
- In addition to noticeable swollen lymph nodes, other symptoms may be present, including:
What causes Castleman disease?
Castleman disease occurs when there is abnormal growth of the type of white blood cell known as lymphocytes. However, the exact reasons for this abnormal growth are not well understood. Elevated levels of a chemical associated with the normal immune response, known as interleukin-6 (IL-6), often is found in people with multicentric Castleman disease. People with multicentric Castleman disease often are found to have a virus known as human herpesvirus-8 (HHV-8), also known as Kaposi sarcoma herpesvirus (KSHV). HHV-8 is often found in the lymph node cells in people with multicentric Castleman disease, especially in those who are HIV positive. This may be the reason for the elevated levels of IL-6.
HHV-8 has not been seen in all cases of multicentric Castleman disease, and many people become infected with HHV-8 and do not develop Castleman disease, so the exact relationship between the viral infection and the condition is not fully understood.
Is Castleman disease inherited (genetic)?
No, Castleman disease is not considered to be an inherited condition.
What are the types of Castleman disease?
The two types of Castleman disease are 1) unicentric, and 2) multicentric Castleman disease, as described previously.
What are the stages of Castleman disease?
Since Castleman disease is not a cancer, there is not a formal staging system (the stage of a cancer refers to the extent to which it has spread in the body). The two types - multicentric and unicentric (see above) - refer to the extent of disease in the body.
What is the prevalence of Castleman disease?
Castleman disease is rare, and there are no reliable estimates of its frequency in the population.
Is there a test to diagnose Castleman disease?
The diagnosis of Castleman disease is made by a tissue sampling (biopsy) of an affected lymph node. The tissue sample is examined by a pathologist (a physician specialized in the diagnosis of diseases from tissue samples) under a microscope, and additional special tests may be done on the tissue sample. A number of blood tests that evaluate immune function may also be performed, but the diagnosis itself depends upon identifying the abnormal lymph node tissue.
What is the treatment for Castleman disease?
Treatment for Castleman disease can involve a combination of different approaches, including:
- Surgery to remove involved lymph nodes (see surgery section)
- Medications to reduce the abnormal immune response (see medication section)
- Radiation therapy to destroy areas of affected lymph nodes that cannot be removed surgically
What medications treat Castleman disease?
Several different classes of medications have been used to treat Castleman disease.
- Corticosteroid drugs suppress the immune response. They can be useful in people with immune system disorders and some cancers. These drugs may be given alone, or sometimes in combination with the type of chemotherapy drugs used to treat cancers.
- These chemotherapy drugs are most often used in Castleman disease
- Chemotherapy and corticosteroids may also be combined with radiation therapy to destroy the abnormal tissue.
- Immunotherapy involves administering drugs that boost or strengthen the body's natural immune response. Immunotherapy drugs include
- Immunomodulating drugs such as thalidomide (Thalomid) and lenalidomide (Revlimid) are used to treat multiple myeloma and some lymphomas, and also can be helpful for some people with Castleman disease.
- Interferon-alfa (a man-made version of an immune-boosting substance produced by the body) is occasionally used to treat Castleman disease.
- Anti-viral medications such as ganciclovir, valganciclovir, and foscarnet have been successfully used to treat multicentric Castleman disease associated with infection with the virus HHV-8.
Daily Health News
Is there surgery to treat Castleman disease?
Surgical removal of the involved lymph nodes is a helpful way to treat unicentric (localized) Castleman disease. Surgery also may be used in multicentric Castleman disease as a way to control symptoms, although in multicentric Castleman disease, it is not typically possible to remove all of the affected lymph nodes.
Which specialties of health-care professionals treat Castleman disease?
A number of different specialists may be involved in the care of a patient with Castleman disease, depending upon the type and extent of the disease, including hematologist-oncologists, radiation therapy specialists, and surgeons. If the patient has HIV-associated Castleman disease, infectious disease specialists will also likely be involved in the patient's care.
What questions should I ask the doctor about Castleman disease?
Questions to ask the doctor about Castleman disease include the following:
- What type of Castleman disease do I have?
- How was the diagnosis established? Was my biopsy read by a pathologist who is an expert on Castleman disease? If not, should I have a second opinion?
- What additional tests, if any, do I need?
- Do you have experience treating patients with Castleman disease? Should I get a second opinion from another physicians regarding treatment options?
- What are all the treatment options, and which do you recommend?
- What are the side effects of treatment, and how will treatment affect my daily life?
- What is the likelihood that the treatment will be successful? If the disease returns after treatment, what are my options?
- What is my prognosis, or outlook for survival?
- What kind of follow-up care will be required after treatment?
- Am I eligible for any clinical trials or new treatments?
What is the life expectancy and prognosis for a person with Castleman disease?
It is difficult to determine the outlook for any given individual with Castleman disease because the condition takes different forms and is very rare. Estimates of 3-year survival rates are based on whether the affected person is also infected with HIV and upon certain characteristics of the lymph nodes when observed under the microscope. For example, in a group of patients with unicentric Castleman disease who had certain microscopic features in their lymph nodes, 93% were alive 3 years after the diagnosis. In HIV+ patients with multicentric disease, only 28% were alive after 3 years. It is important to note that these survival statistics were based upon a small number of patients who were treated in the past, and treatments are always improving.
Health Solutions From Our Sponsors
Radhakrishnan, N., MD. Castleman Disease." Medscape. Updated: Jan 25, 2015.
American Cancer Society. "Castleman Disease." Updated: Jan 27, 2016.
Castleman Disease - Experience
Please share your experience with Castleman disease.Post View 1 Comment
Castleman Disease - Symptoms and Signs
What signs and symptoms have you, or someone you know experienced with Castleman disease?Post
Castleman Disease - Type
What type of Castleman disease do you, or someone you know have? Please share your experience.Post
Castleman Disease - Treatment
Please share what treatments your, or someone you know have had for Castleman disease, and were they helpful?Post
Top Castleman Disease Related Articles
Aches, Pain, FeverAlthough a fever technically is any body temperature above the normal of 98.6 F (37 C), in practice, a person is usually not considered to have a significant fever until the temperature is above 100.4 F (38 C). Fever is part of the body's own disease-fighting arsenal; rising body temperatures apparently are capable of killing off many disease-producing organisms.
Acquired Immunodeficiency Syndrome (AIDS)AIDS is the advanced stage of HIV infection. Symptoms and signs of AIDS include pneumonia due to Pneumocystis jiroveci, tuberculosis, toxoplasmosis, seizures, weakness, meningitis, yeast infection of the esophagus, and Kaposi's sarcoma. Anti-retroviral therapy (HAART) is used in the treatment of AIDS.
ChemotherapyChemotherapy is the treatment of cancer with drugs that can destroy cancer cells. These drugs often are called "anticancer" drugs. Chemotherapy is often used with other treatments. Coping with side effects (fatigue, nausea, vomiting, pain, hair loss, infection, diarrhea, constipation, fluid retention, mouth and throat problems) are important to understand when undergoing chemotherapy treatment. It is important to eat well during chemotherapy, and get the support you need both during and after treatment.
Chronic cough is a cough that does not go away and is generally a symptom of another disorder such as asthma, allergic rhinitis, sinus infection, cigarette smoking, GERD, postnasal drip, bronchitis, pneumonia, medications, and less frequently tumors or other lung disease.
Chronic cough treatment is based on the cause, but may be soothed natural and home remedies.
Oral and injectable systemic corticosterois are steroid hormones prescribed to decrease inflammation in diseases and conditions such as arthritis (rheumatoid arthritis, for example), ulcerative colitis, Crohn's disease, asthma, bronchitis, some skin rashes, and allergic or inflammatory conditions that involve the nose and eyes. Examples of systemic corticosteroids include hydrocortisone (Cortef), cortisone, prednisone (Prednisone Intensol), prednisolone (Orapred, Prelone), and methylprednisolone (Medrol, Depo-Medrol, Solu-Medrol). Some of the side effects of systemic corticosteroids are swelling of the legs, hypertension, headache, easy bruising, facial hair growth, diabetes, cataracts, and puffiness of the face.
cyclophosphamideCyclophosphamide (Cytoxan) is a drug prescribed for the treatment of certain cancers including breast cancer, leukemia, and ovarian cancer. Side effects, drug interactions, patient safety information, and pregnancy and breastfeeding information should be reviewed prior to taking any medication.
Fatigue can be described in various ways. Sometimes fatigue is described as feeling a lack of energy and motivation (both mental and physical). The causes of fatigue are generally related to a variety of conditions or diseases, for example, anemia, mono, medications, sleep problems, cancer, anxiety, heart disease, and drug abuse.Treatment of fatigue is generally directed toward the condition or disease that is causing the fatigue.
Human Immunodeficiency Virus (HIV)The human immunodeficiency virus (HIV) causes HIV infection and the acquired immunodeficiency syndrome (AIDS). Symptoms and signs of HIV infection include fatigue, enlarged lymph glands, and recurrent vaginal yeast infections. Highly active antiretroviral therapy (ART) is the standard treatment for HIV infection.
interferonInterferons are a family of natural occurring proteins. Interferons are used to treat many diseases that involve the immune system for example, cancers, hepatitis, AIDS, multiple sclerosis (MS), genital and perianal warts, and granulomatous disease. Side effects, drug interactions, warnings and precautions and patient safety information should be reviewed prior to taking any medication.
Lymph Nodes PictureAlso sometimes referred to as lymph glands, lymph nodes are small rounded or bean-shaped masses of lymphatic tissue surrounded by a capsule of connective tissue. See a picture of Lymph Nodes and learn more about the health topic.
Nausea and Vomiting
Nausea is an uneasiness of the stomach that often precedes vomiting. Nausea and vomiting are not diseases, but they are symptoms of many conditions. There are numerous cases of nausea and vomiting. Some causes may not require medical treatment, for example, motion sickness, and other causes may require medical treatment by a doctor, for example, heart attack, lung infections, bronchitis, and pneumonia.
Some causes of nausea and vomiting may be life threatening, for example, heart attack, abdominal obstruction, and cancers.
Treatment of nausea and vomiting depends upon the cause.
Night sweats are severe hot flashes that occur at night and result in a drenching sweat. The causes of night sweats in most people are not serious, like menopause in women, sleep apnea, medications, alcohol withdrawal, and thyroid problems. However, more serious diseases like cancer and HIV also can cause night sweats. Your doctor will treat your night sweats depending upon the cause.
You may experience other signs and symptoms that are associated with night sweats, which depend upon the cause, but may include, shaking, and chills with a fever caused by an infection like the flu or pneumonia; unexplained weight loss due to lymphoma; women in perimenopause or menopause may also have vaginal dryness, mood swings, and hot flashes during the day; and low blood sugar in people with diabetes.
Other causes of night sweats include medications like NSAIDs (aspirin, acetaminophen, ibuprofen (Motrin, Advil), and naproxen (Aleve, Naprosyn), antidepressants, sildenafil (Viagra), and abuse of prescription or illegal drugs and drug withdrawal; hormone disorders like pheochromocytoma and carcinoid syndrome; idiopathic hyperhidrosis; infections like endocarditis, AIDs, and abscesses; alcoholism and alcohol withdrawal; drug abuse, addiction, and withdrawal; and stroke.
A doctor or other health care professional can treat your night sweats after the cause has been diagnosed.
Non-Hodgkins LymphomasNon-Hodgkin's lymphoma is cancer of the lymphatic system, a vital part of the body's immune system. Symptoms and signs include swollen lymph nodes, fever, night sweats, coughing, weakness, chest pain, unexplained weight loss, and abdominal pain. Treatment depends on which type of non-Hodgkin's lymphoma one has, the stage of the cancer, one's age, how fast the cancer is growing, and whether one has other health problems.
Radiation TherapyRadiation therapy is a cancer-fighting technique. In radiation therapy, a radiation oncologist uses high-energy rays to kill cancer cells and stop them from growing and dividing. The two types of radiation therapy are external and internal. Potential side effects of radiation therapy include:
- skin redness,
- permanent pigmentation,
- diarrhea, and
- a reduction in white blood cells.
rituximabRituximab (Rituxan) is a medication prescribed for the treatment of non-Hodgkin's B-cell lymphomas, chronic lymphocytic leukemia, granulomatosis with polyangiitis, and microscopic polyangiitis.
Rituxan is combined with methotrexate (Trexall) to treat rheumatoid arthritis. Side effects, drug interactions, dosage, and pregnancy safety should be reviewed prior to taking this medication.
Swollen Lymph NodesLymph nodes help the body's immune system fight infections. Causes of swollen lymph nodes (glands) may include infection (viral, bacterial, fungal, parasites). Symptoms of swollen lymph nodes vary greatly, but may include fever, night sweats, toothache, sore throat, or weight loss. Causes of swollen lymph nodes also vary, but may include cancer, the common cold, mono, chickenox, HIV, and herpes. The treatment of swollen lymph nodes depends upon the cause.