Table 5.

Treatment options

iMCD-NOS and iMCD-TAFROPOEMS-associated MCDHHV8-MCD
First-line therapy   
 • Siltuximab • If no bone lesions, iMCD-like therapy • If HIV-positive, combination antiretroviral therapy 
 • Tocilizumab • If bone lesions, myeloma type therapy including ASCT • Rituximab 
 • Corticosteroids   
Second line and beyond options   
 • Rituximab As above  • Etoposide 
 • Cyclosporin*  • Liposomal doxorubicin 
 • Sirolimus*  • Interferon 
 • IVIG*  • Antiviral therapy 
 • Thalidomide*   
 • Lenalidomide*   
 • Bortezomib   
 • R-CVP,† R-CHOP   
 • ASCT   
iMCD-NOS and iMCD-TAFROPOEMS-associated MCDHHV8-MCD
First-line therapy   
 • Siltuximab • If no bone lesions, iMCD-like therapy • If HIV-positive, combination antiretroviral therapy 
 • Tocilizumab • If bone lesions, myeloma type therapy including ASCT • Rituximab 
 • Corticosteroids   
Second line and beyond options   
 • Rituximab As above  • Etoposide 
 • Cyclosporin*  • Liposomal doxorubicin 
 • Sirolimus*  • Interferon 
 • IVIG*  • Antiviral therapy 
 • Thalidomide*   
 • Lenalidomide*   
 • Bortezomib   
 • R-CVP,† R-CHOP   
 • ASCT   

IVIG, IV immunoglobulin; R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone; R-CVP, rituximab, cyclophosphamide, vincristine, prednisone.

*

Immunomodulatory therapies.

Combination chemotherapies.

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