From the Guidelines
The first-line treatment for HHV-8-associated multicentric Castleman disease (MCD) should be rituximab, potentially combined with etoposide to avoid a possible flare, as evidenced by the latest clinical practice guidelines 1. When considering the treatment of Castleman disease, it's crucial to differentiate between unicentric and multicentric forms, as well as the presence of HHV-8 association.
- For HHV-8-associated MCD, the use of rituximab is recommended, with the option to add etoposide, especially to mitigate the risk of flare 1.
- The dosage and administration route of rituximab can vary, but a common approach is 375 mg/m² weekly for 4 weeks.
- Etoposide, if used, can be administered orally or intravenously, with the intravenous route offering optimized bioavailability 1.
- It's also important to consider the patient's immune status, particularly the CD4 count, when initiating rituximab therapy, as patients with a CD4 count <50 cells/ml may require cautious management 1.
- Regular monitoring and adjustment of treatment based on the patient's response and the burden of disease, such as the presence of Kaposi sarcoma, are critical for effective management 1.
From the Research
Treatment Options for Castleman Disease
- Complete surgical resection is often curative and is therefore the preferred first-line therapy for unicentric Castleman disease (UCD), if possible 2, 3.
- The management of unresectable UCD is more challenging, and existing evidence supports that asymptomatic unresectable UCD may be observed 2, 3.
- The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome 2, 3.
- Unresectable UCD that is symptomatic due to compression of vital neighboring structures may be rendered amenable to resection by medical therapy (e.g., rituximab, steroids), radiotherapy, or embolization 2, 3.
Medical Therapy for Castleman Disease
- Rituximab, an anti-CD20 monoclonal antibody, has been used as a neoadjuvant treatment to enable a less morbid surgical resection in patients with unicentric Castleman disease 4.
- Interleukin-6 (IL-6) has been shown to play a significant role in the pathogenesis of the disease, and anti-IL-6-directed therapies are highly effective in many idiopathic multicentric Castleman disease (iMCD) patients 5, 6.
- Other treatment options for Castleman disease include single agent chemotherapy, combination chemotherapy, interferon (IFN)-alpha, anti-IL-6 receptor antibodies, and thalidomide, although variable benefit has been achieved with these treatments 5.
Classification and Diagnosis of Castleman Disease
- Castleman disease (CD) includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), human herpes virus-8 (HHV8)-associated MCD (HHV8-MCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD (POEMS-MCD) 6.
- The diagnosis and classification of Castleman disease have been improved with the development of international evidence-based consensus diagnostic and treatment guidelines 2, 3, 6.