Table 1.

Clinicopathologic characteristics of patients with concurrent or sequential MN and AITL diagnosis

PatientsSexMNClonal relationshipAge (MN), yAge (AITL), yInterval between MN and AITLBiopsy site and DNA source (MN)Percent AITL involvementBiopsy site and DNA source (AITL)MN treatmentsAITL treatmentsSurvival from AITL diagnosis (mo)Reference
Patients with MN-AITL with paired NGS sequencing (n = 15) 
1 Male CMML Related 76 78 16 months PB Not involved#  LN allo-HSCT BV-CHP, allo-HSCT Alive (21) MSKCC 
2 Male CMML Related 50 55 60 months BM Not involved#  LN allo-HSCT BV-CHP, allo-HSCT Alive (29) MSKCC 
3 Male MDS Related 75 75 Synchronous BM (FC sorted) 2.1% by FC BM (FC sorted) EPOCH Supportive care Deceased (0.2) 5  
4 Female MDS Related 80 86 72 months BM NA LN Not treated CHOP, azacitidine Deceased (20) 6  
5 NA PV, post-PV MF Related NA NA NA BM NA LN NA NA NA 7  
6 Female PV Related 84 86 24 months PB NA LN Phlebotomy, hydroxyurea BV Deceased (12) 8  
7 Male MDS Related 78 81 42 months BM Not involved∗  LN NA NA NA 9  
8 Male CMML Related 83 83 Synchronous BM ∼5%∗  LN NA NA NA 9  
9 Female MDS Related 73 73 Synchronous BM Not involved∗  LN NA NA NA 9  
10 Male PMF Related 64 64 Synchronous BM NA LN Not treated CHOP Deceased (64) 10  
11 Female ET Related 73 76 31 months BM NA LN Supportive care CHOP Deceased (98) 10  
12 Male ET Related 60 65 58 months BM NA LN Supportive care CHOEP, ASCT Deceased (72) 10  
13 Female PV Unrelated 72 76 42 months BM NA LN Phlebotomy, hydroxyurea Supportive care Deceased (1) 10  
14 Female MPN-U NAΔ  62 62 Synchronous BM Involved∗  LN Not treated CHOEP, ASCT Alive (NA) 10  
15 Male CMML Related 70 70 Synchronous BM Not involved##  LN Azacitidine Azacitidine, romidepsin Alive (7) 11  
Patients with AITL-MN with paired NGS sequencing (n = 10) 
16 Male MDS Related 76 75 10 months BM 0.014% by FC LN Azacitidine R-CHOP, ruxolitinib, duvelisib Deceased (17) MSKCC 
17 Male CMML Related 76 73 32 months BM 0.036% by FC LN NA Lenalidomide-CHOEP, ASCT, and cerdulatinib Deceased (61) MSKCC 
18 Male MDS Related 79 74 66 months BM 1% by FC LN Luspatercept, romiplostim CHOP, ASCT, romidepsin, bortezomib, duvelisib, ruxolitinib, and valemetostat Deceased (109) MSKCC 
19 Female AML, therapy related Unrelated 83 80 34 months BM 0.1% by FC Tonsil Azacitidine, venetoclax, Mini-CHOP, duvelisib, romidepsin, and TTI-621 Deceased (78) MSKCC 
20 Female MPN (favored CNL) Related 75 72 36 months BM 0.004% by FC LN Not treated CHOP, duvelisib, romidepsin, bortezomib, BV, and valemetostat Deceased (43) MSKCC 
21 Male MDS Unrelated 56 55 14 months PB 0.03% by FC LN Supportive care CHOEP, ASCT Deceased (16) MSKCC 
22 Male AML
MECOM rearranged 		Related 75 72 46 months BM Not involved by FC LN Azacitidine CHOEP, romidepsin lenalidomide, and carfilzomib Deceased (58) MSKCC 
23 NA CMML Related NA NA NA BM NA LN NA NA NA 7  
24 Male MDS Related 67 64 36 months BM NA LN Azacitidine CHOEP, ASCT Deceased (48) 12  
25 Male AML
NPM1 mutated 		Related 46 45 12 months BM NA LN FLAIE CHOEP, ASCT Deceased (18) 3  
PatientsSexMNClonal relationshipAge (MN), yAge (AITL), yInterval between MN and AITLBiopsy site and DNA source (MN)Percent AITL involvementBiopsy site and DNA source (AITL)MN treatmentsAITL treatmentsSurvival from AITL diagnosis (mo)Reference
Patients with MN-AITL with paired NGS sequencing (n = 15) 
1 Male CMML Related 76 78 16 months PB Not involved#  LN allo-HSCT BV-CHP, allo-HSCT Alive (21) MSKCC 
2 Male CMML Related 50 55 60 months BM Not involved#  LN allo-HSCT BV-CHP, allo-HSCT Alive (29) MSKCC 
3 Male MDS Related 75 75 Synchronous BM (FC sorted) 2.1% by FC BM (FC sorted) EPOCH Supportive care Deceased (0.2) 5  
4 Female MDS Related 80 86 72 months BM NA LN Not treated CHOP, azacitidine Deceased (20) 6  
5 NA PV, post-PV MF Related NA NA NA BM NA LN NA NA NA 7  
6 Female PV Related 84 86 24 months PB NA LN Phlebotomy, hydroxyurea BV Deceased (12) 8  
7 Male MDS Related 78 81 42 months BM Not involved∗  LN NA NA NA 9  
8 Male CMML Related 83 83 Synchronous BM ∼5%∗  LN NA NA NA 9  
9 Female MDS Related 73 73 Synchronous BM Not involved∗  LN NA NA NA 9  
10 Male PMF Related 64 64 Synchronous BM NA LN Not treated CHOP Deceased (64) 10  
11 Female ET Related 73 76 31 months BM NA LN Supportive care CHOP Deceased (98) 10  
12 Male ET Related 60 65 58 months BM NA LN Supportive care CHOEP, ASCT Deceased (72) 10  
13 Female PV Unrelated 72 76 42 months BM NA LN Phlebotomy, hydroxyurea Supportive care Deceased (1) 10  
14 Female MPN-U NAΔ  62 62 Synchronous BM Involved∗  LN Not treated CHOEP, ASCT Alive (NA) 10  
15 Male CMML Related 70 70 Synchronous BM Not involved##  LN Azacitidine Azacitidine, romidepsin Alive (7) 11  
Patients with AITL-MN with paired NGS sequencing (n = 10) 
16 Male MDS Related 76 75 10 months BM 0.014% by FC LN Azacitidine R-CHOP, ruxolitinib, duvelisib Deceased (17) MSKCC 
17 Male CMML Related 76 73 32 months BM 0.036% by FC LN NA Lenalidomide-CHOEP, ASCT, and cerdulatinib Deceased (61) MSKCC 
18 Male MDS Related 79 74 66 months BM 1% by FC LN Luspatercept, romiplostim CHOP, ASCT, romidepsin, bortezomib, duvelisib, ruxolitinib, and valemetostat Deceased (109) MSKCC 
19 Female AML, therapy related Unrelated 83 80 34 months BM 0.1% by FC Tonsil Azacitidine, venetoclax, Mini-CHOP, duvelisib, romidepsin, and TTI-621 Deceased (78) MSKCC 
20 Female MPN (favored CNL) Related 75 72 36 months BM 0.004% by FC LN Not treated CHOP, duvelisib, romidepsin, bortezomib, BV, and valemetostat Deceased (43) MSKCC 
21 Male MDS Unrelated 56 55 14 months PB 0.03% by FC LN Supportive care CHOEP, ASCT Deceased (16) MSKCC 
22 Male AML
MECOM rearranged 		Related 75 72 46 months BM Not involved by FC LN Azacitidine CHOEP, romidepsin lenalidomide, and carfilzomib Deceased (58) MSKCC 
23 NA CMML Related NA NA NA BM NA LN NA NA NA 7  
24 Male MDS Related 67 64 36 months BM NA LN Azacitidine CHOEP, ASCT Deceased (48) 12  
25 Male AML
NPM1 mutated 		Related 46 45 12 months BM NA LN FLAIE CHOEP, ASCT Deceased (18) 3  

allo-HSCT, allogeneic hematopoietic stem cell transplantation; AML, acute myeloid leukemia; ASCT, autologous stem cell transplantation; BM, bone marrow; BV, brentuximab vedotin; BV-CHP, brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisolone; CHOEP, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, etoposide, and prednisone; CMML, chronic myelomonocytic leukemia; CNL, chronic neutrophilic leukemia; EPOCH, etoposide, prednisolone, vincristine, cyclophosphamide, and hydroxydaunorubicin; ET, essential thrombocythemia; FC, flow cytometric study; FLAIE, fludarabine, cytarabine, etoposide, idarubicin; LN, lymph node; MDS, myelodysplastic syndrome; MF, myelofibros; MPN, myeloproliferative neoplasm; MPN-U, myeloproliferative neoplasm, unclassifiable; NA, not available; PB, peripheral blood; PMF, primary myelofibrosis; PV, polycythemia vera; R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

#

NGS studies for MNs in cases #1 and #2 were performed on samples obtained at the time of initial MN diagnosis, which occurred several years prior to the development of AITL. As a result, the MN samples were uninvolved by AITL based on flow cytometric and morphologic analysis.

∗

The extent of bone marrow involvement by AITL was assessed by review of histomorphology and immunohistochemical staining.

##

The methodology to assess AITL involvement was not specified.

∆

We excluded this patient from clonal relatedness assessment due to ambiguous genetic findings: the bone marrow sample was involved by AITL (extent of involvement was not specified in the original paper); the bone marrow and AITL samples shared only an IDH2 mutation with a 2% VAF in both samples and a RHOA mutation was only reported in the bone marrow but not in the AITL sample.

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