MULTICENTRIC CASTLEMAN DISEASE (DISSEMINATED)
Multicentric or “disseminated” CD is found in both adults and children.
It is not a form of cancer but it can be serious.
Discovery
The disease is usually detected by signs of inflammation: fever, sweats, weight loss, failure to thrive in children or unexplained anaemia.
Lymph nodes (adenopathies) can be felt at the side of the neck, the underarm or groin area. In some cases, the spleen may be enlarged (splenomegaly)
Diagnosis
A diagnosis is made after examining a sample (biopsy) taken from a lymph node.
This sample may be taken by a surgeon (minor operation under local anaesthesia) or by a radiologist who inserts a needle into the lymph node.
The lesions characteristic of Castleman disease can be detected by analysing this sample. The doctor in charge of this analysis (anatomopathologist) will then indicate or confirm the diagnosis.
Initial assessment
Based on the biopsy report which indicates this diagnosis, the doctor in charge will propose various tests to confirm the diagnosis.
- The doctor will be systematically looking for anaemia and signs of inflammation by examining a blood sample. The patient will also be screened for HIV infection. With this form of Castleman disease, the examinations will usually reveal anaemia, signs of inflammation with raised C-reactive protein (CRP) and gammaglobulin levels. This inflammation is linked to the production of a protein called interleukin 6 or IL-6
- A radiological examination of the whole body (scan or PET scan) can reveal the affected lymph nodes and investigate the spleen.
Treatment
The reference treatment involves an antibody (immunotherapy) which neutralizes the effect of the IL-6.
Two antibodies may be used subject to availability: siltuximab (Sylvant®) and tocilizumab (RoActemra®). This treatment is suspensive and its duration is therefore indeterminate. It produces rapid effects but relapse is common if the treatment is withdrawn.
Corticosteroids or another antibody, rituximab (Mabthera®) may be used to treat less severe forms.
Outcome
The prognosis has improved significantly since the introduction of these new treatments but serious, resistant, life-threatening forms still exist.