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Table 1 Demographic data, clinical data, and characteristics of reported cases of IVL of T and NK-cell lineage

From: Intravascular NK/T-cell lymphoma, Epstein–Barr virus positive with multiorgan involvement: a clinical dilemma

Ref. Age/sex Sites involved at presentation/(symptoms/ signs) Sites involved during disease course BOM involvement Immunophenotype EBER TCR Therapy/outcome
Santucci 2003 [2] 54/M Skin lesions, weight loss, leukopenia CNS NA CD3e + CD56 + TIA-1 + GrB + CD30 + ki671 + 100%LMP1-CD4- CD8- CD20- CD79a- CD57- CD68- Bcl2- + NA CHOP/Exitus 17 mo after diagnosis
Wu 2005 [3] 41/M Skin lesions none CD3e + CD2 + CD7 + CD56 + CD43 + perforin+ TIA-1 + Bcl2 + CD20- CD4- CD8- CD5- TCRbF1- CD30- lysozyme- MPO- Keratin- + TCR germline CHOP+
SCT
CR at 12 mo
Wu 2005 [3] 47/F Fever, weakness, arthralgia, myalgia, mental signs+ pancytopenia Disseminated disease at autopsy (brain, bone marrow, kidneys, ovaries, cervix) + (diagnosis made on BOM biopsy) CD3e + CD2 + CD7 + CD56 + GrB + TIA-1 + CD20- CD5- CD4- CD8- CD57- TCR germline Unspecified therapy/exitus 15 days after diagnosis
Kuo 2006 [4] 71/F Skin lesions none CD3e + CD56 + TIA1 + KI67 + 90%CD4- CD5- CD8- CD10- CD20-CD30- BCL6- LMP-1- + TCR germline No therapy/alive at 5 mo
Song 2007 40/F Skin lesions+CNS none LCA + CD3 + CD56+ GrB + TIA + 1+ Ki67 + 100%
CD20- CD4- CD8- CD29-
+ TCR germline CODOX-M + IVAC/alive at 7mo
Nakamichi 2008 [6] 23/F Skin lesions Ileum, spleen+fever CD3e + CD56+
TIA1 + ki67 + 100%CD20- CD79a- CD45RO-
+ TCR germline CHOP followed by PromMACE/CYTABOM, L-ASP/CY, hyper CVAD/MTX-AraC, SCT. Exitus after 9 mo, due to aGVHD
Cerroni 2008 [7] 67/F Skin lesion+CNS none NA CD2+ CD3e + CD8 + CD56-TIA-1 + CD4- CD20- CD30+/- CD56- NA TCR monoclonal (TCRg) Exitus after 7 days
Cerroni 2008 [7] 63/M Skin lesions+arthralgias, weight loss, fever, depression+leukopenia+ thrombocytopenia none NA CD2 + CD3e + TIA1 + CD56+ CD4- CD5- CD7- CD8- CD20- CD45RO + bF1- + TCR germline Exitus after 6 mo
Cerroni 2008 [7] 64/M (History of B-CLL). Skin lesions NA - (B-CLL+) CD2 + CD3e + TIA-1 + CD4- CD5- CD8- CD20- CD56- bF1- TCR germline CHOP, exitus after 7 mo
Cerroni 2008 [7] 87/M Skin lesions+portal hyperthension none NA CD2 + CD3e + CD56- TIA-1- GrB-perforin- CD4- CD5- CD7- CD8-CD20- CD30- CD45RO- bF1- + TCR monoclonal (TCRg) Exitus after 15 days
Gleason 2008 [8] 62/M Skin lesions+night sweats none LCA + CD2 + CD3e + CD43 + CD56 + Perforin+ GrB + TIA-1 + CD4- CD5- CD7- CD8- CD20- CD79a- BCL2- CD30- CD45RO- TCRbF1- TdT- MPO- CK- CD34- TCR monoclonal(TCRg) CHOP followed by DHAP. AWD at 8 mo
Liao 2011 [11] 42/F Skin lesions+ malaise, dizziness. none CD3e + CD56 + Gr-B + KI67 + 99%,bF1- CD4- CD5- CD8- CD20- CD30- PAX5- TdT- + NA RT + CT: (CHOP,Bortezomib, EPOCH); alive at 14 mo
Yanning 2013 84/F Skin lesions+fever, weight loss NA NA CD2 + CD3e + CD45RO + GrB + CD4- CD5- CD7- CD8- CD20- CD79a- CD56- CD30- panCK- + TCR germline Therapy refused. AWD at 4 mo
Jang 2014 23/F Skin lesions none CD3 + CD8 + GrB + TIA-1 + LCA + MPO + CD4- CD5- CD20- CD30- CD56- CK- + TCR germline
IgH gene clonal
CHOP+intratecal MTX + PBSCT.
Skin recurrence followed by unspecified CT. Exitus 11 mo after recurrence
Liu 2014 38/F Skin lesions CNS CD3 + CD56 + GrB + Ki67 + 90%CD4- CD5- CD8- CD20- CD30- PAX5- TdT- + NA CHOP. After 7 mo recurrence at CNS. Exitus 13 mo after diagnosis
Wang 2015 45/M Skin lesions+malaise, fever, weight loss NA NA CD2 + CD3e + perforin+ GrB + TIA1 + CD56 + CD4- CD8- CD20- TCRb- TCRg- CD30- KI67 + 90–100% + TCR germline Exitus 15 days after diagnosis
Wang 2015 32/F Skin lesions+fever none NA CD2 + CD3e + perforin+ GrB + TIA1 + CD56 + CD4- CD8- CD20- TCRb- TCRg- CD30- KI67 + 90–100% + TCR germline CHOP. Exitus 4 mo after diagnosis
Wang 2015 18/F Skin lesions none CD2 + CD3e + perforin+ GrB + TIA1 + CD56 + CD4- CD8- CD20- TCRb- TCRg- CD30 + KI67 + 90–100% + TCR germline CHOP. Alive 3 yrs. after diagnosis
Bi 2015 29/M Skin lesions, fever, weight loss Liver CD3 + CD43 + CD56 + TIA-1 + CD30 + CD4- CD5- CD7- CD8- CD20- CD79a- KI67 + 90% + TCR germline HyperCVAD. Exitus 3 mo after diagnosis
Alhumidi 2015 48/F Skin lesions None CD45 + CD3 + GrB + CD56- CD4- CD5- CD8- CD20- + NA CT. Alive 18 mo after diagnosis
Jaffe 2017 51/M Skin lesions+CNS none NA CD3 + CD8 + TIA-1 + GrB + CD20- CD79a- CD56- ALK-1- + TCR monoclonal (TCR-g) NA
Sharma 2017 62/F CNS none + on molecular analysis (TCR) CD3 + CD8 + CD57 + GrB + TIA-1 + CD2- CD4- CD5- CD7- CD30- CD56- TdT- PAX5- TCR monoclonal Methylprednisolone, dexamethasone, intratechal MTX, cytarabine. Exitus
Alegria-Landa 2017 81/M Skin lesions none CD3 + GrB + perforin+ CD30 + CD20- CD4- CD8- TCRb- TCRg- CD56- + TCR monoclonal (TCRbTCRg) Exitus 15 days after diagnosis
Present case 54/M Jaundice, fever, respiratory symptoms, pancytopenia. PB: no atypical lymphocytes Disseminated disease at autopsy (brain, heart, kidney, lung, bone marrow) + (subtle infiltration) CD3 + CD2 + perforin+ GrB + CD56 + KI67 + 80% CD20- CD79a- PAX5- CD4- CD8- CD5- ALK1-CD16- + TCR monoclonal Exitus 18 days after presentation
  1. Ffemale, M male, BOM bone marrow, NA not assessed, CR complete remission, mo months, yrs. years, B-CLL B chronic lymphocytic leukemia, RT radiotherapy, CT chemotherapy, CHOP cyclophosphamide, doxorubicin, vincristine, prednisone, SCT Stem cell transplant, CODOX-M cyclophosphamide, vincristine, doxorubicin, methotrexate, IVAC ifosfamide, mesna, etoposide, cytarabine, ProMACE/CytaBOM prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide, cytosine, arabinoside, bleomycin, vincristine, leucovorin, l-ASP l-asparaginase, CY cyclophosphamide, hyper-CVAD hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, MTX methotrexate, AraC cytosine arabinoside, aGVHD acute graft-versus-host disease, EPOCH etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, DHAP dexamethasone, cytarabine, cisplatin, PBSCT peripheral blood stem-cell transplant, PB peripheral blood, AWD alive with disease