Novel treatments in clinical trials including MZL patients with relapsed/refractory NHL or relapsed BCL
| Pathway . | Drug . | Target . | No. of evaluable MZL patients* . | Response . | Limiting toxicity . | |
|---|---|---|---|---|---|---|
| Name . | Reference . | |||||
| PI3K/AKT/mTOR | Everolimus | 59 | mTOR | 24 MZL (16 MALT; 4 NMZL) | CR 4%-ORR 28% | Hematologic (46% G3/4), interstitial pneumonia (4%) |
| Idelalisib | 60 | PI3K-d | 15 MZL | CR 6%-ORR 57% | Neutropenia (27% G3/4), aminotransferase elevations (13% G3/4), diarrhea (13% G3/4), pneumonia (7% G3/4) | |
| Copanlisib | 61 | PI3K-d and PI3K-α | 3 MZL | ORR 2/3 | Hypertension (49% G3/4), neutropenia (30% G3/4), hyperglycemia (30% G3/4), anemia (15% G3/4) | |
| BCR | Ibrutinib | 62 | BTK | 4 MZL | ORR 1/4 | Diarrhea (< 5% G3/4), neutropenia (13% G3/4), thrombocytopenia (7% G3/4), anemia (7% G3/4) |
| Apoptosis | ABT-199 | 63 | BCL2 | 3 MZL | Unknown for MZL | Neutropenia (27%), anemia (19%), thrombocytopenia (19%), febrile neutropenia (12%) |
| Microenvironment | Lenalidomide | 64 | Immunomodulator | 40 MZL | CR 65% ORR 86% | Neutropenia (35%), muscle pain (9%), rash (7%), cough, dyspnea, or other pulmonary symptoms (5%), thrombosis (5%), thrombocytopenia (4%) |
| Proteasome | Bortezomib | 65,66 | Proteasome | 48 MALT | CR 35% ORR 64% | Peripheral neuropathy (65%) |
| Pathway . | Drug . | Target . | No. of evaluable MZL patients* . | Response . | Limiting toxicity . | |
|---|---|---|---|---|---|---|
| Name . | Reference . | |||||
| PI3K/AKT/mTOR | Everolimus | 59 | mTOR | 24 MZL (16 MALT; 4 NMZL) | CR 4%-ORR 28% | Hematologic (46% G3/4), interstitial pneumonia (4%) |
| Idelalisib | 60 | PI3K-d | 15 MZL | CR 6%-ORR 57% | Neutropenia (27% G3/4), aminotransferase elevations (13% G3/4), diarrhea (13% G3/4), pneumonia (7% G3/4) | |
| Copanlisib | 61 | PI3K-d and PI3K-α | 3 MZL | ORR 2/3 | Hypertension (49% G3/4), neutropenia (30% G3/4), hyperglycemia (30% G3/4), anemia (15% G3/4) | |
| BCR | Ibrutinib | 62 | BTK | 4 MZL | ORR 1/4 | Diarrhea (< 5% G3/4), neutropenia (13% G3/4), thrombocytopenia (7% G3/4), anemia (7% G3/4) |
| Apoptosis | ABT-199 | 63 | BCL2 | 3 MZL | Unknown for MZL | Neutropenia (27%), anemia (19%), thrombocytopenia (19%), febrile neutropenia (12%) |
| Microenvironment | Lenalidomide | 64 | Immunomodulator | 40 MZL | CR 65% ORR 86% | Neutropenia (35%), muscle pain (9%), rash (7%), cough, dyspnea, or other pulmonary symptoms (5%), thrombosis (5%), thrombocytopenia (4%) |
| Proteasome | Bortezomib | 65,66 | Proteasome | 48 MALT | CR 35% ORR 64% | Peripheral neuropathy (65%) |
BCL, B-cell lymphoma; CR, complete remission; G, grade; NHL, non-Hodgkin lymphoma.
The number of patients with MALT, SMZL, and NMZL was not systematically available.