Castleman's disease is a rare disorder characterized by non-cancerous (benign) growths (tumors) that may develop in the lymph node tissue throughout the body (i.e., systemic disease [plasma cell type]). Most often, they occur in the chest, stomach, and/or neck (i.e., localized disease [hyaline-vascular type]). Less common sites include the armpit (axilla), pelvis, and pancreas. Usually the growths represent abnormal enlargement of the lymph nodes normally found in these areas (lymphoid hamartoma). There are two main types of Castleman's disease: hyaline-vascular type and plasma cell type. The hyaline vascular type accounts for approximately 90 percent of the cases. Most individuals exhibit no symptoms of this form of the disorder (asymptomatic) or they may develop non-cancerous growths in the lymph nodes. The plasma cell type of Castleman's disease may be associated with fever, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/or abnormally increased amounts of certain immune factors in the blood (hypergammaglobulinemia).
A third type of Castleman's disease has been reported in the medical literature. This type may affect more than one area of the body (multicentric or generalized Castleman's disease). Many individuals with Multicentric Castleman's disease may exhibit an abnormally large liver and spleen (hepatosplenomegaly). Researchers' opinions in the medical literature differ as to whether Multicentric Castleman's disease is a distinct entity or a multicentric form of the plasma cell type of Castleman's disease. .
In most cases, Castleman's disease is characterized by a single, solid growth within lymphatic tissue in the chest, stomach, or neck. Growths may also occur in other lymphatic tissue throughout the body. Usually the growths represent abnormal enlargement of the lymph nodes (lymphoid hamartoma) normally found in these areas. There are three types of Castleman's disease: hyaline-vascular type; plasma cell type; and a third type that affects more than one area of the body (generalized or multicentric Castleman's) has been also been identified.
In most cases of the hyaline-vascular type of Castleman's disease, individuals exhibit no symptoms of this type of the disorder (asymptomatic) or may develop a non-cancerous (benign) growth in the lymph tissue; most frequently in the chest. Symptoms with this type are usually secondary to the size and location of the growth. For example, a growth may form in a vein, resulting in a bulge and possible obstruction in the involved blood vessel.
In the plasma cell type of Castleman's disease, individuals may exhibit a variety of symptoms including fever, fatigue, excessive sweating, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/or abnormally elevated amounts of certain immune factors in the blood (hypergammaglobulinemia).
Multicentric or generalized Castleman's disease affects many areas of the body. Individuals with this type of Castleman's disease often exhibit symptoms similar to those associated with the plasma cell type. In addition, some individuals may have an enlarged liver and spleen (hepatosplenomegaly). Some cases of multicentric Castleman's disease have been associated with POEMS syndrome. (For more information on this disorder, choose "POEMS" as your search term in the Rare Disease Database.) Researchers have speculated that individuals with this type of Castleman's disease may have a greater risk of developing malignant complications such as Kaposi's sarcoma or malignant lymphoma. .
The exact cause of Castleman's disease is not known. Some researchers speculate that increased production of interleukin-6 (IL-6) may be involved in the development of Castleman's disease. IL-6 is a substance produced by structures within the lymph nodes. Recently, a virus has been found to be the cause of some cases of multicentric Castleman's disease. Human herpesvirus 8 (also known as HHV-8, Kaposi's sarcoma-associated herpesvirus, or KSHV) is found in nearly all patients with human immunodeficiency virus (HIV) who develop multicentric Castleman's disease and up to 80% of patients without HIV. .
Castleman's disease is a rare disorder of the lymphatic system that affects males and females in equal numbers. All types of Castleman's disease may affect individuals of any age; however, the plasma cell type has greater prevalence among young males and females. Children are rarely affected by this disorder. Persons with HIV are at increased risk of developing multicentric Castleman's disease. .
Symptoms of the following disorders can be similar to those of Castleman's disease. Comparisons may be useful for a differential diagnosis:
Hodgkin's disease is a form of cancer of the lymphatic system. Tumors occur in the lymph nodes and/or the areas around the nodes. Symptoms associated with this disorder may include fever, night sweats, weight loss, and/or enlarged or swollen lymph nodes. The tumors occur most often in the chest, stomach, or spleen. Hodgkin's disease is usually progressive and may spread to involve lymph nodes located in other areas of the body. The exact cause of Hodgkin's disease is not known. (For more information on this disorder, choose "Hodgkin" as your search term in the Rare Disease Database.)
The following disorders may be associated with Castleman's disease as secondary characteristics. They are not necessary for a differential diagnosis:
Malignant lymphoma is a general term for a group of lymphatic tumors. Malignant lymphomas generally occur in the chest and/or stomach. Symptoms common to all forms of malignant lymphomas include fevers, excessive sweating at night, weight loss, and/or an abnormally enlarged liver and/or spleen (hepatosplenomegaly).
Kaposi's sarcoma is a malignant skin tumor that may spread to other parts of the body. Affected individuals may have skin lesions (e.g., papules, plaques, etc.) that may grow and come together (coalesce). In some cases, the lesions may reduce in size and number (regress). In addition, on rare occasions these lesions may be painful. .
The diagnosis of Castleman's disease may 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 abnormal growths associated with Castleman's disease. Identifying the presence of elevated levels of interleukin-6 may also assist a diagnosis of Castleman's disease, as can the detection of human herpesvirus-8 among patients with the mullticentric variant.
The treatment of Castleman's Disease is directed toward the specific symptoms that are apparent in each individual. For localized disease, surgical removal of the affected lymph node(s) usually results in cure. Specific therapies for the treatment of this disorder are symptomatic and supportive. Surgical removal (excision) of the growth is the preferred treatment in most cases of localized Castleman's Disease. In some cases, ionizing radiation (radiotherapy) has proven effective. For multicentric disease, chemotherapy and radiation are the cornerstones of treatment. Additional symptomatic and supportive therapy may include corticosteroids or blood transfusions. Corticosteriods have been used to treat specific symptoms that may be associated with the plasma cell and multicentric types of this disorder. .
As of June of 2008 an ongoing clinical research study is enrolling patients with Castleman's disease. The purpose of this study is to evaluate different doses and schedules of CNTO 328 to see which dose/schedule is safe. This research study will use a type of drug called anti-IL-6 antibody, also known as CNTO 328. This study is being conducted at 10 centers in the United States. For more information, go to www.clinicaltrials.gov (identifier: NCT00412321).
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted by the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
For information about clinical trials sponsored by private sources, contact: www.centerwatch.com
The drug, Actemra, manufactured by Chugai Pharmaceuticals in Japan, has been approved for the treatment of Castleman's disease in Japan. It is still an investigational product in the United States. Chugai Pharmaceuticals has a satellite office in New Jersey and can be contacted at the following:
One Crossroads Drive Building A, 2nd Floor Bedminster, NJ 07921-2688 Phone: (908) 947-2700
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Madisons Foundation PO Box 241956 Los Angeles, CA 90024 Tel: (310)264-0826 Fax: (310)264-4766 Email: getinfo@madisonsfoundation.org Internet: http://www.madisonsfoundation.org
UCSF Hemophilia Treatment Center 400 Parnassus Ave. First Floor San Francisco, CA 94143 Tel: (415)353-2986 Fax: (415)353-2600 Internet: http://www.ucsfhealth.org/adult/medical_services/blood/hemophilia/index.html
International Castleman's Disease Organization 4 Brazos Court Santa Fe, NM 87508 Tel: (505)424-7947 Fax: (505)424-7948 Email: HMConsult@msn.com Internet: http://www.castlemans.org
AutoImmunity Community Tel: (919) 552-9057 Email: bandrews@autoimmunitycommunity.org Internet: http://autoimmunitycommunity.org
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