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NCCN Guidelines for Castleman Disease V.1.2025

Request Number Guideline Page Specific change request Panel Decision Comment Institution Vote
4766 - Internal Request CD-C 1 of 3 First-line therapy

MCD - Criteria for active disease present but no organ failure
HHV8-negative/HIV-1-negative (iMCD -Nonsevere) 
•  Add: RCVP (rituximab, cyclophosphamide, vincristine, prednisone)

MCD - Fulminant/severe ± organ failure
Fulminant/severe HHV8-negative (iMCD)
•  Add the following: 
• CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) ± rituximab
• CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) ± rituximab
• CVP (cyclophosphamide, vincristine, prednisone) ± rituximab
• Siltuximab
• If not a candidate for combination therapy: Rituximab

 Fulminant/severe HHV8-positive MCD
•  Add the following: 
• Rituximab + liposomal doxorubicin
• Rituximab + prednisone + liposomal doxorubicin

Change made Yes: 33
No: 0
Abstain:0
Absent:1
4767 - Internal Request CD-C 2 of 3 Second-Line and Subsequent Therapy for Relapsed/Refractory or Progressive Disease

HHV8(+) - Criteria for active disease present with no organ failure or Fulminant/Severe disease [HHV8(+) or HHV8(-)] with no organ failure
Preferred regimens
• Add: Siltuximab (if HHV8-negative)
Other recommended regimens
• Add: Sirolimus

Fulminant/Severe disease [HHV8(+) or HHV8(-)] with organ failure
Preferred regimens
Add:  Siltuximab (if HHV8-negative)
Change made Yes: 33
No: 0
Abstain:0
Absent:1