In Castleman disease, non-cancerous growths (tumors) develop in lymph node tissue in the body. Castleman disease affects both males and females, and may occur at any age, but typically it does not affect children. Little is known about how often Castleman disease actually occurs, other than it is rare, or what causes the disease.

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.

Castleman's disease belongs to the group of atypical lymphoid proliferations, which are usually confused with the diagnosis of malignant lymphoma. First described in 1956, the typical patient presents with either localized mediastinal lymphadenopathy or a more aggressive form of the disease characterized by diffuse lymphadenopathy and systemic symptoms

Castleman's disease, also known as angiofollicular lymph node hyperplasia, is a rare disorder with three identified histologic variants (hyaline vascular-type, plasma cell-type, and mixed-type) and two clinical types (localized and multicentric). Only occasional cases of mixed-type disease have been reported.1, 2 Clinically, in contrast to the localized form, multicentric Castleman's disease is a systemic illness that frequently presents with general signs, polyadenopathy, organomegaly, and occasionally POEMS syndrome.

Multicentric Castleman disease (MCD) is a rare disorder that causes numerous problems, including fatigue, fever, anemia, and tumor-like growths in multiple lymph nodes. Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8, is found in about 50% of MCD cases not associated with HIV, and in nearly 100 percent of HIV-associated MCD. Some patients may also have Kaposi sarcoma.

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).

Causes

Doctors are still trying to determine the exact cause of Castleman disease, but many suspect infection by a virus called the human herpes virus 8 (HHV8). This virus has been associated with Kaposi sarcoma, a cancerous tumor of the blood vessel walls. It's not uncommon for a person with multicentric Castleman disease to also have Kaposi sarcoma, especially if the person is also HIV-positive.

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.


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Autor: peterhutch

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