MULTICENTRIC CASTLEMAN DISEASE ASSOCIATED WITH THE HHV-8 VIRUS AND HIV
Multicentric CD associated with the HHV-8 virus found in patients infected with HIV is similar to the disease not associated with HIV (see specific sheet).
However, it is often more serious and if left untreated, it can develop rapidly into a serious or even life-threatening form.
Discovery
If HIV infection is known and treated, the disease is detected by signs of inflammation: fever, sweats, weight loss, severe unexplained anaemia or the appearance of lymph nodes (adenopathies). The spleen may be very enlarged (splenomegaly).
In half of all cases, it exists alongside another complication of the HHV-8 infection, namely Kaposi’s sarcoma, which produces violet lesions in the skin.
In some cases, HIV infection has not been diagnosed and it will come to light as a predispositon for the Castleman disease.
Diagnosis
A diagnosis is made after examining a sample (biopsy) taken from a lymph node. The lesions characteristic of Castleman disease can be detected by analysing this sample and the HHV-8 virus can be detected in the lymph node using a specific technique. HHV-8 can also be found in the blood using a technique known as PCR.
Initial assessment
Based on the biopsy report which indicates this diagnosis, the doctor in charge will propose various tests to confirm the diagnosis.
In addition to the assessment of the Castleman disease, an evaluation of HIV infection must also be carried out:
- Identification of further complications, particularly infections
- Identification of lesions typical of Kaposi’s sarcoma
- Measurement of the HIV viral load
- Measurement of the CD4 lymphocyte count.
Treatment
The reference treatment involves an antibody (immunotherapy) which destroys the cells (B lymphocytes) which contain the HHV-8 virus. The antibody used is rituximab (Mabthera®). It is often associated with treatment with etoposide (vepeside®, celltop®) in an emergency, which quickly brings the symptoms under control.
An effective antiretroviral treatment is essential in order to obtain and maintain a good response to the treatment.
Relapse is possible but the disease generally responds to a second treatment.
Outcome
The prognosis has improved significantly since the introduction of these new treatments.
There is a risk of the disease developing into a malignant lymph node tumour (lymphoma), but this risk has been significantly reduced since the introduction of rituximab®.