NCCN Guidelines for B-Cell Lymphomas V.1.2020 – Interim – 12/18/19

AIDS-Related B-cell Lymphomas

Guideline Page Institution Vote Panel Discussion/References

and Request YES NO ABSTAIN ABSENT AIDS-4 Internal request Based on the review of data in the noted reference, the panel 15 5 3 6 Comment to consider the consensus was to include bortezomib-ICE +/- as an option inclusion of bortezomib-ICE +/- for relapsed/refractory HIV-positive DLBCL. This was added a rituximab as an option for category 2B recommendation. relapsed/refractory HIV-positive • Reid EG, Looney D, Maldarelli F, et al. Safety and efficacy of anoncolytic DLBCL. viral strategy using bortezomib with ICE/R in elapsed/refractory HIV- positive lymphomas. Blood Adv 2018;2:3618-3626.tory

Post-Transplant Lymphoproliferative Disorders (PTLD)

Guideline Page Institution Vote Panel Discussion/References

and Request YES NO ABSTAIN ABSENT PTLD-A 21 0 2 6 Internal request Based on the review of data in the noted reference, the panel Comment to consider the consensus was to include + CHP inclusion of brentuximab vedotin (cyclophosphamide, doxorubicin, prednisone) as an option for CD30+ + CHP (cyclophosphamide, monomorphic PTLD (T-cell type). This was added a category 2A doxorubicin, prednisone) for recommendation. CD30+ monomorphic PTLD (T- • Horwitz S, O'Connor OA, Pro B, et al. Brentuximab vedotin with cell type). chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON- 2): a global, double-blind, randomised, phase 3 trial. Lancet 2019;393:229-240. PTLD-A Internal request The panel consensus supported the inclusion of high-dose 20 0 3 6 Comment to consider the methotrexate + rituximab as an option for primary CNS PTLD (B-cell inclusion of high-dose type). methotrexate + rituximab for primary CNS PTLD (B-cell type).

NCCN Guidelines for B-Cell Lymphomas V.1.2020 – Interim – 12/18/19

Castleman’s Disease (CD)

Guideline Page Institution Vote Panel Discussion/References

and Request YES NO ABSTAIN ABSENT CD-3 External request Based on the discussion of data in the references provided the panel 19 1 3 6 Submission request from discussion was to list siltuximab as a preferred regimen for the primary EUSA Pharma, Inc. the treatment of HIV-1, negative/HHV-8 negative/idiopathic multicentric guidelines recommend rituximab Castleman’s disease (plasmacytic/mixed histology). and siltuximab as first-line options. Published clinical data and expert consensus guidance • See Submission for references. full story based on extensive evidence supports siltuximab as a preferred intervention over rituximab for the treatment of herpesvirus-8 (HHV-8)/idiopathic Multicentric (iMCD); please consider siltuximab as a preferred intervention for iMCD. CD-3 Internal request Based on the review of data in the noted reference, the panel 16 1 6 6 Comment to consider the consensus was to include thalidomide, cyclophosphamide, and inclusion of thalidomide, prednisone as a primary treatment option for HIV-1, negative/HHV-8 cyclophosphamide, prednisone negative/idiopathic multicentric Castleman’s disease (hyaline vascular as a primary treatment option for histology). This was added as a category 2A, other recommended HIV-1, negative/HHV-8 option. negative/idiopathic multicentric • Zhang et al. Phase 2 study using oral thalidomide-cyclophosphamide- Castleman’s disease (hyaline prednisone for idiopathic multicentric Castleman disease. Blood vascular histology). 2019;133:1720-1728.