**6. Management**

Treatment modalities for CD include supportive care and specific modalities of treatment.

In the unicentric form of the disease, surgical resection of the localised site of the disease is usually curative. However, follow up is recommended as patients may rarely relapse or develop complications (such as an increased risk of lymphoma development) [23,26,28].

For MCD, a variety of specific treatment options are available, in addition to supportive care (such as analgesia, allopurinol, transfusion of blood and blood products where indicated). Specific treatment modalities include antiviral (anti-herpesvirus) and antiretroviral drugs where a viral association is documented, corticosteroids, monoclonal antibodies, immunomo‐ dulatory agents (such as thalidomide), splenectomy, and radiotherapy [29].

Chemotherapy has evolved from single-agent (e.g. chlorambucil) to combination chemother‐ apy (cyclophosphamide, doxorubicin, vincristine, prednisone – CHOP), to the addition of rituximab and etoposide [3,11,20,29,30,31]. Bower, 2010 [29], in his excellent review on 'How I treat HIV-associated multicentric Castleman disease', uses a combination of weekly IVI rituximab (375 mg/m2 ) with IVI etoposide (100 mg/m2 ) for 4 weeks for aggressive HIVassociated MCD, with an overall 2 year survival of 85% and rituximab monotherapy (375 mg/ m2 weekly for 4 weeks) for low-risk HIV-associated MCD, with an overall 2 year survival of 100% [29].

For HIV-negative, HHV-8 negative MCD, other therapeutic options have been explored. This includes monoclonal antibodies directed against IL-6 (siltuximab) or the IL-6 receptor (tocili‐ zumab) [27].

Multicentric Castleman's disease (MCD) is increasingly being recognised as a relapsing and remitting disease and in the HIV-seropositive setting is not necessarily suppressed or dimin‐ ished by combination antiretroviral therapy [29]. As such, the role of both rituximab and antiherpesvirus agents such as valganciclovir have been explored as maintenance therapies [20,29,32,33]. However, the role of maintenance therapy in this disease remains controversial and requires further evaluation.
