Characteristics of lymphomas, treatment, and outcome in 5 IL10R deficient patients
| Patient . | Lymphoma . | Age at onset (years) . | IS at lymphoma onset . | Organ involvement . | Treatment . | Outcome (post HSCT) . |
|---|---|---|---|---|---|---|
| P1 | P1-L1 | 5.8 | 0 | Liver, spleen, retroperitoneal and intra-abdominal LN, bone | COP, COPADM × 2, CYM × 2 | Remission of lymphoma, EBV-related LPD at 10.3 y, died of progression |
| P2 | P2-L1 | 5.3 | Azathioprine | Liver, bone | COP, R-COPADM | Died during treatment |
| P3 | P3-L1 | 6.5 | Azathioprine | Liver, intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2, HSCT | Remission, alive (+12 mo) |
| P4 | P4-L1 | 5.5 | 0 | Spleen, pelvis, and inguinal LN | COP, COPADM × 2, CYM × 2 | |
| P4-L2 | 9.7 | 0 | Spleen, mediastinal, and intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2 | ||
| P4-L3 | 12.1 | 0 | Spleen, mediastinal, and intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2 | ||
| P4-L4 | 16.5 | 6MP and MTX | Spleen, mediastinal, and intra-abdominal LN | R-ICE × 4, HSCT | Remission, alive (+18 mo) | |
| P5 | P5-L1 | 6.5 | 0 | Abdominal LN | Up-front R prephase,* (2A4-2B4) × 4 | Remission, alive |
| Patient . | Lymphoma . | Age at onset (years) . | IS at lymphoma onset . | Organ involvement . | Treatment . | Outcome (post HSCT) . |
|---|---|---|---|---|---|---|
| P1 | P1-L1 | 5.8 | 0 | Liver, spleen, retroperitoneal and intra-abdominal LN, bone | COP, COPADM × 2, CYM × 2 | Remission of lymphoma, EBV-related LPD at 10.3 y, died of progression |
| P2 | P2-L1 | 5.3 | Azathioprine | Liver, bone | COP, R-COPADM | Died during treatment |
| P3 | P3-L1 | 6.5 | Azathioprine | Liver, intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2, HSCT | Remission, alive (+12 mo) |
| P4 | P4-L1 | 5.5 | 0 | Spleen, pelvis, and inguinal LN | COP, COPADM × 2, CYM × 2 | |
| P4-L2 | 9.7 | 0 | Spleen, mediastinal, and intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2 | ||
| P4-L3 | 12.1 | 0 | Spleen, mediastinal, and intra-abdominal LN | COP, R-COPADM × 2, R-CYM × 2 | ||
| P4-L4 | 16.5 | 6MP and MTX | Spleen, mediastinal, and intra-abdominal LN | R-ICE × 4, HSCT | Remission, alive (+18 mo) | |
| P5 | P5-L1 | 6.5 | 0 | Abdominal LN | Up-front R prephase,* (2A4-2B4) × 4 | Remission, alive |
2A4, dexamethasone, vincristine, cytarabine, etoposide, methotrexate, ifosfamide; 2B4, dexamethasone, vincristine, doxorubicin, methotrexate, cyclophosphamide;6MP, 6 mercaptopurine; BM, bone marrow; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; COP, cyclophosphamide, vincristine, prednisone; COPADM, cyclophosphamide, vincristine, prednisone, doxorubicin, methotrexate; CYM, cytarabine, methotrexate; ICE, ifosfamide, carboplatin, etoposide; IS, immunosuppressive therapy; LN, lymph node; LPD, lymphoproliferative disease; MTX, methotrexate; R, rituximab.
Prephase, dexamethasone and cyclophosphamide.