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