Clinicopathologic characteristics of patients with concurrent or sequential MN and AITL diagnosis and incomplete molecular information
| Patients . | Sex . | MN . | Clonal relationship . | Age (MN), y . | Age (AITL), y . | Interval between MN and AITL . | Molecular results . | MN treatments . | AITL treatments . | Survival from AITL diagnosis (mo) . | Reference . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Patients with MN-AITL with partial sequencing, paired Sanger sequencing, or no sequencing (n = 14) | |||||||||||
| 26 | Female | MPN | NA | 59 | 71 | 138 months | JAK2 p.V617F was detected in the MN by JAK2 single gene assay. 2 TET2 mutations detected in AITL by NGS. | Pegylated interferon, EPO mimetic | CEP | Deceased (23) | MSKCC |
| 27 | Male | AML | NA | 57 | 79 | 264 months | Not performed | HiDAC/IDR, cytarabine, and etoposide | Supportive care | Deceased (2) | MSKCC |
| 28 | Female | MDS | NA | 72 | 72 | 6 months | Not performed | NA | CHOP, pralatrexate, vorinostat, and BV | Deceased (64) | MSKCC |
| 29 | Female | CMML | Related | 70 | 80 | 120 months | Same TET2 mutation detected in both MN and AITL by Sanger sequencing DNMT3A, IDH2, and RHOA G17V were not detected in either neoplasm. | Not treated initially, later azacytidine | Azacytidine, rituximab | Alive (18) | 13 |
| 30 | Female | AML NPM1 mutation | NA | 65 | 75 | 120 months | TET2 mutation detected in MN No molecular data for AITL | Cytarabine, anthracycline, ASCT | BV-CHP | Alive (6) | 14 |
| 31 | Female | ET | NA | 78 | 78 | 6 months | Not performed | Hydroxyurea | CHOEP, R-ICE | Alive (5) | 15 |
| 32 | Male | ET | NA | 78 | 84 | 72 months | JAK2 p.V617F detected in MN No molecular data for AITL | Hydroxyurea | CEOP | Deceased (4) | 15 |
| 33 | Male | AML RUNX1::RUNX1T1 | NA | 47 | 47 | 4 months | Not performed | Idarubicin, cytarabine | Cyclophosphamide, vincristine, prednisolone | Deceased (1) | 16 |
| 34 | NA | CMML | NA | 55 | 56 | 7 months | Not performed | Hydroxyurea | MOPP, CHOP | Deceased (22) | 17 |
| 35 | Female | CMML | NA | 86 | 86 | Synchronous | Mutations in TET2, DNMT3A, ASXL1, SRSF2, NRAS, IDH1, CSF3R detected in MN by NGS No molecular data for AITL. | Azacytidine | Azacytidine, vinblastine | Alive (18) | 18 |
| 36 | NA | MDS | Related | NA | NA | NA | Same TET2 and DNMT3A mutations detected in both MN and AITL by Sanger sequencing. | NA | NA | NA | 19 |
| 37 | NA | MDS | Related | NA | NA | NA | Same DNMT3A mutation detected in both MN and AITL by Sanger sequencing. TET2 mutation detected only in AITL | NA | NA | NA | 19 |
| 38 | NA | MPN | NA | 58 | 62 | 48 months | Not performed | NA | NA | NA | 2 |
| 39 | NA | MDS | NA | 73 | 76 | 36 months | Not performed | NA | NA | NA | 2 |
| Patients with MN-AITL with partial sequencing, paired Sanger sequencing, or no sequencing (n = 2) | |||||||||||
| 40 | Female | AML | NA | 78 | 77 | 7 months | No molecular data for MN Negative for RHOA p.G17V and IDH2 p.R172 mutations in AITL by Sanger sequencing | NA | CHOP | Deceased (7) | 20 |
| 41 | Female | AML KMT2A rearranged | NA | 60 | 58 | 18 months | Not performed | Decitabine, cytarabine, and idarubicin | R-EPOCH, ASCT | Alive (21) | 21 |
| Patients . | Sex . | MN . | Clonal relationship . | Age (MN), y . | Age (AITL), y . | Interval between MN and AITL . | Molecular results . | MN treatments . | AITL treatments . | Survival from AITL diagnosis (mo) . | Reference . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Patients with MN-AITL with partial sequencing, paired Sanger sequencing, or no sequencing (n = 14) | |||||||||||
| 26 | Female | MPN | NA | 59 | 71 | 138 months | JAK2 p.V617F was detected in the MN by JAK2 single gene assay. 2 TET2 mutations detected in AITL by NGS. | Pegylated interferon, EPO mimetic | CEP | Deceased (23) | MSKCC |
| 27 | Male | AML | NA | 57 | 79 | 264 months | Not performed | HiDAC/IDR, cytarabine, and etoposide | Supportive care | Deceased (2) | MSKCC |
| 28 | Female | MDS | NA | 72 | 72 | 6 months | Not performed | NA | CHOP, pralatrexate, vorinostat, and BV | Deceased (64) | MSKCC |
| 29 | Female | CMML | Related | 70 | 80 | 120 months | Same TET2 mutation detected in both MN and AITL by Sanger sequencing DNMT3A, IDH2, and RHOA G17V were not detected in either neoplasm. | Not treated initially, later azacytidine | Azacytidine, rituximab | Alive (18) | 13 |
| 30 | Female | AML NPM1 mutation | NA | 65 | 75 | 120 months | TET2 mutation detected in MN No molecular data for AITL | Cytarabine, anthracycline, ASCT | BV-CHP | Alive (6) | 14 |
| 31 | Female | ET | NA | 78 | 78 | 6 months | Not performed | Hydroxyurea | CHOEP, R-ICE | Alive (5) | 15 |
| 32 | Male | ET | NA | 78 | 84 | 72 months | JAK2 p.V617F detected in MN No molecular data for AITL | Hydroxyurea | CEOP | Deceased (4) | 15 |
| 33 | Male | AML RUNX1::RUNX1T1 | NA | 47 | 47 | 4 months | Not performed | Idarubicin, cytarabine | Cyclophosphamide, vincristine, prednisolone | Deceased (1) | 16 |
| 34 | NA | CMML | NA | 55 | 56 | 7 months | Not performed | Hydroxyurea | MOPP, CHOP | Deceased (22) | 17 |
| 35 | Female | CMML | NA | 86 | 86 | Synchronous | Mutations in TET2, DNMT3A, ASXL1, SRSF2, NRAS, IDH1, CSF3R detected in MN by NGS No molecular data for AITL. | Azacytidine | Azacytidine, vinblastine | Alive (18) | 18 |
| 36 | NA | MDS | Related | NA | NA | NA | Same TET2 and DNMT3A mutations detected in both MN and AITL by Sanger sequencing. | NA | NA | NA | 19 |
| 37 | NA | MDS | Related | NA | NA | NA | Same DNMT3A mutation detected in both MN and AITL by Sanger sequencing. TET2 mutation detected only in AITL | NA | NA | NA | 19 |
| 38 | NA | MPN | NA | 58 | 62 | 48 months | Not performed | NA | NA | NA | 2 |
| 39 | NA | MDS | NA | 73 | 76 | 36 months | Not performed | NA | NA | NA | 2 |
| Patients with MN-AITL with partial sequencing, paired Sanger sequencing, or no sequencing (n = 2) | |||||||||||
| 40 | Female | AML | NA | 78 | 77 | 7 months | No molecular data for MN Negative for RHOA p.G17V and IDH2 p.R172 mutations in AITL by Sanger sequencing | NA | CHOP | Deceased (7) | 20 |
| 41 | Female | AML KMT2A rearranged | NA | 60 | 58 | 18 months | Not performed | Decitabine, cytarabine, and idarubicin | R-EPOCH, ASCT | Alive (21) | 21 |
AML, acute myeloid leukemia; ASCT, autologous stem cell transplantation; BV, brentuximab vedotin; BV-CHP, brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisolone; CEOP, cyclophosphamide, etoposide, vincristine, and prednisone; CEP, cyclophosphamide, etoposide, and prednisone; CHOEP, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, etoposide, and prednisone; CMML, chronic myelomonocytic leukemia; EPOCH, etoposide, prednisolone, vincristine, cyclophosphamide, and hydroxydaunorubicin; ET, essential thrombocythemia; HiDAC/IDR, high-dose cytarabine, idarubicin; MDS, myelodysplastic syndrome; MOPP, methylchloretamine, vincristine, procarbazine, and prednisone; MPN, myeloproliferative neoplasm; NA, not available; R-ICE, rituximab, ifosfamide, carboplatin and etoposide.