Castelman`s disease

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Overview

Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD was first described in the 1950s by Benjamin Castleman as localized mediastinal lymph node enlargement characterized by increased numbers of lymphoid follicles with germinal center involution and marked capillary proliferation, including follicular and interfollicular endothelial hyperplasia.1  In 1969, Flendrig described the plasma cell (PC), the hyalinized, and the “intermediate” (or mixed) histopathological variants.2,3  Further descriptions over the years provided insight into clinicopathologic associations.3,4  By the mid-1980s, CD was divided into unicentric CD (UCD), which involved a single enlarged lymph node or region of lymph nodes, and multicentric CD (MCD), which involved multiple lymph node stations.5,6  Investigators also noted an association between HIV and MCD.7,8  Co-occurrence with and overlap between the PC neoplasm polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome (also known as Takatsuki or Crow-Fukase) and MCD was also noted in the 1980s and 1990s; later, the monoclonal PCs causing POEMS were proposed to be causing the MCD in these cases. Human herpes virus-8 (HHV8) was identified as the etiological driver of all HIV+ and some HIV− MCD cases in the 1990s. In the 2010s, Takai et al recognized a severe form of HHV8− or idiopathic MCD (iMCD) in which patients had a homogeneous constellation of abnormal laboratory tests and clinical features that he called thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly (TAFRO) syndrome.9,10  Recently, the Castleman Disease Collaborative Network (CDCN) proposed a classification system retaining the UCD vs MCD nomenclature, but further dividing MCD by etiological driver (HHV8-associated MCD [HHV8-MCD]; POEMS-associated MCD [POEMS-MCD]; iMCD) and within iMCD by phenotype, iMCD-TAFRO, and iMCD–not otherwise specified 

Symptoms

Signs and symptoms of Castleman disease vary depending on the type. People with unicentric Castleman disease (UCD) do not always have symptoms. Doctors usually discover the disease during an exam for another condition. When symptoms do occur, they include:


Pressure or full feeling in the abdomen (belly) or chest

Lump beneath the skin in the armpit, neck, or groin

Unexplained weight loss

Fever.

Night sweats.

Fatigue (extreme tiredness)

Appetite and weight loss.

Abnormally large lymph nodes, typically in the neck, armpit, collarbone, and groin.

Enlarged spleen or liver.

Anemia (low amount of red blood cells)

Causes

It's not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease. The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor that can be a complication of HIV/AIDS.

Infection such as human herpes virus 8 (HHV-8) and possibly others as well as problems with the body’s immune system may cause Castleman disease. Castleman disease can be associated with other cancers such as lymphoma.

Risk factors

Most patients with Castleman disease (CD) don't have any known risk factors. The only clear risk factor for CD is infection with HIV, the virus that causes AIDS1. The multicentric form of Castleman disease is much more common in people with HIV infection, particularly in those who have developed AIDS.

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Complications

People with Castleman disease have an increased risk of developing cancers including lymphoma (cancer of the lymph system) and Kaposi's sarcoma (a cancerous skin tumor). Some people with MCD develop infections that can damage organs and be life-threatening if they are not treated.

Prevention

You can reduce your risk of Castleman disease by reducing your risk of being infected with HIV. To prevent the spread of this infection you can:


Use condoms during sexual activity

Limit the number of your sexual partners

Avoid drug use

Avoid sharing needles during intravenous (into the vein) drug use