Clinical Data on CD56+ Lymphomas
| Case No. . | Sex/Age . | Major Sites of Disease at Presentation . | EBER In Situ Hybridization . | Treatment . | Outcome . |
|---|---|---|---|---|---|
| Nasal-type NK/T-cell lymphomas | |||||
| 136 | F/63 | Skin | + | Chemotherapy (BACOP, then BVP) | DOD 6 mo |
| 228 | M/47 | Testis | + | Orchiectomy and chemotherapy (MECOP-B) | Developed disease in gastrointestinal tract and nasal cavity. DOD 4 mo |
| 328 | M/71 | Testis, gastrointestinal tract | + | Orchiectomy | DOD 1 wk |
| 428 | M/55 | Testis | + | Orchiectomy | Developed skin lesions and hepatosplenomegaly at 1 mo. Given chemotherapy. DOD 5 mo |
| 527 | M/63 | Salivary gland | + | Excision and chemotherapy (CHOP) | Relapsed in pharynx and palate at 2.5 yr |
| 6 | M/47 | Oropharynx, liver, spleen, marrow | + | Chemotherapy (ProMACE-CytaBOM) | DOD 1 wk |
| 7 | F/21 | Liver (RHS) | + | Nil | DOD 1 month |
| 8 | F/41 | Breast, nasopharynx | + | Excision and chemotherapy (ProMACE-CytaBOM) | Developed periorbital edema, lower limb edema and chest wall nodule. DOD 3 mo |
| 9 | M/39 | Gastrointestinal tract, spleen | + | Colectomy | DOD 3 wk |
| 10 | M/23 | Marrow (RHS) | + | Chemotherapy | DOD 4 mo |
| 11 | F/76 | Skin | + | Chemotherapy (IMVP-16) | Repeated relapses in skin and soft tissues; DOD 3 yr |
| 12 | M/68 | Soft tissues | + | Amputation of limb and chemotherapy (ProMACE-CytaBOM) | AW 3 yr |
| 1334 | M/73 | Soft tissues | + | Chemotherapy (CEOP) and radiotherapy | Relapsed as multiple skin nodules; DOD 9 mo |
| 1434 | M/41 | Lymph nodes, tonsil, marrow (RHS) | + | Chemotherapy (ProMACE-CytaBOM) | DOD 6 mo |
| 1527,34 | M/64 | Salivary gland, tonsil, testis, gastrointestinal tract | + | Chemotherapy (mBACOD) | DOD 5 mo |
| 1637 | M/61 | Marrow, liver, spleen (RHS) | − | High dose steroid | DOD 2 wk |
| 1725,34,36 | F/28 | Skin | + | Chemotherapy (CHOP, then MACOP-B) and radiotherapy | Relapsed in spleen, liver, mediastinum and meninges; DOD 7 mo |
| 1825,27,34 | M/24 | Salivary gland, skin | + | Surgery | DOD 6 wk |
| 1925,34 | F/39 | Jejunum | + | Surgery and chemotherapy (ACOMLA) | Developed pleural effusion; DOD 1 mo |
| 2025 | M/57 | Skin, lymph nodes | + | Chemotherapy (CVP) | DOD 4 mo |
| 21 | M/67 | Skin | + | Information not available | Not available |
| 2236 | M/52 | Skin | + | Information not available | Not available |
| 2336 | M/34 | Skin, maxillary antrum, lymph nodes | + | Radiotherapy | DOD 1 mo |
| 2436 | M/61 | Skin | + | Radiotherapy | Systemic relapse (soft tissue, lungs, spleen, lymph nodes) and complicated by RHS; DOD 1 yr |
| 2536 | F/69 | Skin, lungs, lymph nodes | + | Nil | DOD 1 mo |
| 2636 | F/44 | Skin (RHS) | + | Chemotherapy (vincristine, bleomycin) | DOD 1 mo |
| 27 | M/35 | Lip | − | Chemotherapy (CHOP) | Relapsed in epiglottis at 3 yr; AW at 5 yr |
| 28 | F/30 | Skin | + | Information not available | Not available |
| 29 | M/75 | Skin | + | Information not available | Not available |
| 30 | M/43 | Spleen | + | Information not available | Not available |
| 31 | M/26 | Soft tissue (root of penis) | + | Chemotherapy (ProMACE-CytaBOM) | Relapsed in marrow, spleen and liver, and DOD 6 mo |
| 32 | M/48 | Soft tissue, pancreas, nasopharynx, palate, testis | + | Chemotherapy (M-BACOP) | DOD 7 wk |
| 33 | M/56 | Larynx | + | Chemotherapy (ProMACE-CytaBOM) and radiotherapy | Relapsed in terminal ileum (perforation) and mesenteric lymph nodes at 2 yr |
| 34 | M/67 | Skin | + | Nil (misdiagnosed as vasculitis) | Developed disease in larynx at 5 mo |
| Case No. . | Sex/Age . | Major Sites of Disease at Presentation . | EBER In Situ Hybridization . | Treatment . | Outcome . |
|---|---|---|---|---|---|
| Nasal-type NK/T-cell lymphomas | |||||
| 136 | F/63 | Skin | + | Chemotherapy (BACOP, then BVP) | DOD 6 mo |
| 228 | M/47 | Testis | + | Orchiectomy and chemotherapy (MECOP-B) | Developed disease in gastrointestinal tract and nasal cavity. DOD 4 mo |
| 328 | M/71 | Testis, gastrointestinal tract | + | Orchiectomy | DOD 1 wk |
| 428 | M/55 | Testis | + | Orchiectomy | Developed skin lesions and hepatosplenomegaly at 1 mo. Given chemotherapy. DOD 5 mo |
| 527 | M/63 | Salivary gland | + | Excision and chemotherapy (CHOP) | Relapsed in pharynx and palate at 2.5 yr |
| 6 | M/47 | Oropharynx, liver, spleen, marrow | + | Chemotherapy (ProMACE-CytaBOM) | DOD 1 wk |
| 7 | F/21 | Liver (RHS) | + | Nil | DOD 1 month |
| 8 | F/41 | Breast, nasopharynx | + | Excision and chemotherapy (ProMACE-CytaBOM) | Developed periorbital edema, lower limb edema and chest wall nodule. DOD 3 mo |
| 9 | M/39 | Gastrointestinal tract, spleen | + | Colectomy | DOD 3 wk |
| 10 | M/23 | Marrow (RHS) | + | Chemotherapy | DOD 4 mo |
| 11 | F/76 | Skin | + | Chemotherapy (IMVP-16) | Repeated relapses in skin and soft tissues; DOD 3 yr |
| 12 | M/68 | Soft tissues | + | Amputation of limb and chemotherapy (ProMACE-CytaBOM) | AW 3 yr |
| 1334 | M/73 | Soft tissues | + | Chemotherapy (CEOP) and radiotherapy | Relapsed as multiple skin nodules; DOD 9 mo |
| 1434 | M/41 | Lymph nodes, tonsil, marrow (RHS) | + | Chemotherapy (ProMACE-CytaBOM) | DOD 6 mo |
| 1527,34 | M/64 | Salivary gland, tonsil, testis, gastrointestinal tract | + | Chemotherapy (mBACOD) | DOD 5 mo |
| 1637 | M/61 | Marrow, liver, spleen (RHS) | − | High dose steroid | DOD 2 wk |
| 1725,34,36 | F/28 | Skin | + | Chemotherapy (CHOP, then MACOP-B) and radiotherapy | Relapsed in spleen, liver, mediastinum and meninges; DOD 7 mo |
| 1825,27,34 | M/24 | Salivary gland, skin | + | Surgery | DOD 6 wk |
| 1925,34 | F/39 | Jejunum | + | Surgery and chemotherapy (ACOMLA) | Developed pleural effusion; DOD 1 mo |
| 2025 | M/57 | Skin, lymph nodes | + | Chemotherapy (CVP) | DOD 4 mo |
| 21 | M/67 | Skin | + | Information not available | Not available |
| 2236 | M/52 | Skin | + | Information not available | Not available |
| 2336 | M/34 | Skin, maxillary antrum, lymph nodes | + | Radiotherapy | DOD 1 mo |
| 2436 | M/61 | Skin | + | Radiotherapy | Systemic relapse (soft tissue, lungs, spleen, lymph nodes) and complicated by RHS; DOD 1 yr |
| 2536 | F/69 | Skin, lungs, lymph nodes | + | Nil | DOD 1 mo |
| 2636 | F/44 | Skin (RHS) | + | Chemotherapy (vincristine, bleomycin) | DOD 1 mo |
| 27 | M/35 | Lip | − | Chemotherapy (CHOP) | Relapsed in epiglottis at 3 yr; AW at 5 yr |
| 28 | F/30 | Skin | + | Information not available | Not available |
| 29 | M/75 | Skin | + | Information not available | Not available |
| 30 | M/43 | Spleen | + | Information not available | Not available |
| 31 | M/26 | Soft tissue (root of penis) | + | Chemotherapy (ProMACE-CytaBOM) | Relapsed in marrow, spleen and liver, and DOD 6 mo |
| 32 | M/48 | Soft tissue, pancreas, nasopharynx, palate, testis | + | Chemotherapy (M-BACOP) | DOD 7 wk |
| 33 | M/56 | Larynx | + | Chemotherapy (ProMACE-CytaBOM) and radiotherapy | Relapsed in terminal ileum (perforation) and mesenteric lymph nodes at 2 yr |
| 34 | M/67 | Skin | + | Nil (misdiagnosed as vasculitis) | Developed disease in larynx at 5 mo |
| Aggressive NK cell lymphoma/leukemia | |||||
| 3534 | F/39 | Blood, marrow, lymph nodes, liver, spleen | + | Nil | DOD 4 d |
| 3625,34,39 | M/50 | Blood, marrow, liver, spleen, pleura, skin | + | Prednisone | DOD 1 mo |
| 37 | M/37 | Lymph nodes, blood, marrow, liver | + | Chemotherapy (mBACOD) | DOD 6 wk |
| 3834 | F/41 | Blood, marrow, liver, spleen* | + | Chemotherapy (BACOP, then mBACOP) | Initial partial response, followed by rapid progression; DOD 1 mo |
| 39 | M/54 | Blood, marrow, liver (RHS) | + | Nil | DOD 3 d |
| Blastoid NK cell lymphoma | |||||
| 40 | M/21 | Blood, marrow, tonsils, lymph nodes | − | Chemotherapy (ProMACE-CytaBOM) | Partial response, then changed to German protocol for acute lymphoblastic leukemia, with good response; alive at 10 mo |
| 41 | M/4 | Chin | − | Chemotherapy | Recent case |
| Other specific lymphoma types | |||||
| 4225,34,39 | M/12 | Spleen, lymph nodes, liver, marrow | − | Steroid and splenectomy | DOD 11 wk |
| 4325,34 | M/50 | Skin | − | Chemotherapy (chlorambucil) followed by radiotherapy | DOD 1 yr |
| 4425,34,40 | M/67 | Skin, spleen, blood, marrow | − | Nil | DOD 4 d |
| 4534,41 | M/39 | Spleen, liver, marrow, blood, lymph node | − | Chemotherapy (chlorambucil, COP, then m-BACOP) | DOD 2.5 yr |
| 46 | F/46 | Lymph nodes, marrow | − | German protocol for acute lymphoblastic leukemia | Recent case |
| 47 | F/15 | Soft tissue of face | − | Chemotherapy | AW 4 yr |
| 4825,34 | M/31 | Skin, liver, spleen, lymph nodes | − | Steroids | DOD 1 mo |
| 49 | M/6 mo | Cervical lymph node | − | Chemotherapy | Systemic relapse at 6 mo, and DOD 10 mo |
| Aggressive NK cell lymphoma/leukemia | |||||
| 3534 | F/39 | Blood, marrow, lymph nodes, liver, spleen | + | Nil | DOD 4 d |
| 3625,34,39 | M/50 | Blood, marrow, liver, spleen, pleura, skin | + | Prednisone | DOD 1 mo |
| 37 | M/37 | Lymph nodes, blood, marrow, liver | + | Chemotherapy (mBACOD) | DOD 6 wk |
| 3834 | F/41 | Blood, marrow, liver, spleen* | + | Chemotherapy (BACOP, then mBACOP) | Initial partial response, followed by rapid progression; DOD 1 mo |
| 39 | M/54 | Blood, marrow, liver (RHS) | + | Nil | DOD 3 d |
| Blastoid NK cell lymphoma | |||||
| 40 | M/21 | Blood, marrow, tonsils, lymph nodes | − | Chemotherapy (ProMACE-CytaBOM) | Partial response, then changed to German protocol for acute lymphoblastic leukemia, with good response; alive at 10 mo |
| 41 | M/4 | Chin | − | Chemotherapy | Recent case |
| Other specific lymphoma types | |||||
| 4225,34,39 | M/12 | Spleen, lymph nodes, liver, marrow | − | Steroid and splenectomy | DOD 11 wk |
| 4325,34 | M/50 | Skin | − | Chemotherapy (chlorambucil) followed by radiotherapy | DOD 1 yr |
| 4425,34,40 | M/67 | Skin, spleen, blood, marrow | − | Nil | DOD 4 d |
| 4534,41 | M/39 | Spleen, liver, marrow, blood, lymph node | − | Chemotherapy (chlorambucil, COP, then m-BACOP) | DOD 2.5 yr |
| 46 | F/46 | Lymph nodes, marrow | − | German protocol for acute lymphoblastic leukemia | Recent case |
| 47 | F/15 | Soft tissue of face | − | Chemotherapy | AW 4 yr |
| 4825,34 | M/31 | Skin, liver, spleen, lymph nodes | − | Steroids | DOD 1 mo |
| 49 | M/6 mo | Cervical lymph node | − | Chemotherapy | Systemic relapse at 6 mo, and DOD 10 mo |
Abbreviations: DOD, died of disease; AW, alive and well; RHS, reactive hemophagocytic syndrome.
Immunophenotype reinterpreted as being CD3− CD5− CD7− CD8− instead of CD3+ CD5+ CD7+ CD8+ as originally reported in Tsang et al.34