Castleman's disease

Castleman's disease ( Castleman 's disease ) is defined as hypertrophy of the lymph nodes with angiofollikulärer lymphatic hyperplasia. That said, there are one or more lymph nodes enlarged ( hypertrophy), because the cells of blood vessels ( angio ...) and reaction centers ( follicles ) have proliferated ( hyperplasia). So there are tumors. The cause of the disease is unknown, an investment of HHV - 8 infection is suspected.

There are two forms of M. Castleman distinguished: the localized form in which only one lymph node is infected, and the multicentric form, in which several lymph nodes.

The disease occurs regardless of age and gender. It is estimated that less than 1:100,000 is affected by the disease, with the localized form occurs much more frequently. Most commonly, the abdomen, the superficial lymph nodes and the mediastinum ( mediastinal chest cavity) are affected by the disease. The single lesion can grow to several centimeters in diameter.

M. Castleman was first described in 1954 by the American pathologist Benjamin Castleman.

Symptoms

Causing the localized form at about half of the patients had no complaints. Otherwise, fatigue, fever, weight loss and chest or abdominal pain may occur.

Multicenter progressive forms usually cause symptoms: weight loss ( 69%), fever ( 67 % ), peripheral lymphadenopathy ( 81%), hepatomegaly and / or splenomegaly ( 74%) and not rare [ 23 %] POEMS syndrome ( polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes ). In addition, malignant lymphomas can occur or are already present. In rare cases, Kaposi's sarcoma are described, in the face of the association with HIV and HHV -8 is not surprising. HHV -8 has long been suspected as the causal agent of Kaposisarkome, especially in Koizidenz with severe immune defects such as HIV.

With more than 60 % of all HIV-affected M. Castleman's patients HHV -8 could be detected, while on the other hand HIV negative Castleman's patients only in 20-40 % of cases are HHV -8 positive.

Diagnosis

The diagnosis is made by biopsy with subsequent histological and patho - chemical investigation.

Types

There are two types, which differ histologically and pathogenetically:

The plasma cell-rich variant occurs in particular in the context of HIV disease. In many cases, this can be detected, the human herpes virus 8, which is also blamed for Kaposi's sarcoma. The hyalinized vascular shape is, however, negative for the virus.

Paraneoplastic pemphigus A can often be associated with the M. Castleman.

Treatment

In the localized form of Castleman M. a complete surgical removal must be carried out, resulting in 90% of cases of healing.

In some cases successfully modulated with antiviral drugs or the immune system drugs could be treated. A monoclonal antibody Siltuximab with orphan drug is currently in phase III clinical testing. In March 2014 Siltuximab received a positive CHMP recommendation.

A possible underlying HIV disease treated with antiretroviral drugs. The prognosis of multicentric Castleman M. is bad. She was in a study in HIV -infected patients on average only 14 months.