Table 3.
Clinical features and TCR rearrangements of patients with CTCL-γδ without SPTCL features
| Patient | Age/gender | Primary cutaneous/subsutaneous sites | Extracutaneous sites | Recurrent/relapse sites | TNM | Stage | B symptoms | HPS | Therapy | Follow-up (mo) | TCRγ gene rearrangement | TCRβ gene rearrangement |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 64/M | Head and neck, chest, abdomen, back, R arm, hand (bilateral), leg (bilateral) | Pharynx, L. axillary LN | R inguinal LN | T3bN0M1 | IV | + | − | CHOP, PR→THP-COP for relapse, PD →MINE+ESHAP, PD | DOD, 70 | ND | ND |
| 2 | 22/M | Head, R upper arm, leg(bilateral) | Pharynx | − | T3bN2M1 | IV | + | − | CHOP, steroid, MEPP, NC | DOD, 3 | ND | ND |
| 3 | 23/M | Neck, arm(bilateral), R leg | − | Skin/subcutis (submandible) | T3bN0M0 | IV | − | − | BACOP, CR→CHOP+RT for recurrence, CR→RT, CHOP-B+PBSCT for recurrence, CR→VP-16 for repeating recurrence | AWR, 259 | ND | ND |
| 4 | 79/M | Head and neck | L auricular LN, cervial and supraclavicular, axillary LN (bilateral), R inguinal LN | − | T3bN2M0 | IV | − | − | VP-16, PD | DOD, 8 | ND | ND |
| 5 | 75/M | L face, arm | − | − | T3bN0M0 | IV | − | − | VP-16, INF-γ, PUVA, PD→RT, PD→ACNU, clinical trial(Panobinostat), PD→THP-COP, PD | DOD, 65 | Clonal | Clonal |
| 6 | 72/M | Chest, abdomen and genital, back, arm and leg (bilateral) | Axillary and inguinal LN (bilateral), BM | − | T3bN2M1 | IV | − | − | CPA+UVB, PR | Died of B lymphoblastic leukemia/lymphoma, 28 | UD | Clonal |
| 7 | 54/F | Back, buttocks | R inguinal LN | − | T2N1M0 | II | − | − | Steroid, IFN-γ, PUVA, ACNU, NC→Etretinate, PUVA, NC→RT, CHOP, PUVA, PD→RT, NC→UVB, PD→CHOP, RT, PD→MTX, THP-COP, RT | DOD, 121 | UD | Clonal |
| 8 | 64/M | Head and neck | − | − | T1bN0M0 | I | − | − | No treatment | Died of cerebral hemorrhage, 2 | Clonal | Polyclonal |
| 9 | 50/F | Head | − | Nasal cavity | T2bN0M0 | I | − | − | CHOP, NC→VP-16 for recurrence, PR | AWR, 20 | UD | Polyclonal |
| 10 | 33/M | Trunk and extremities | − | − | ND | IV | + | − | IFN, RT, Steroid, NC | DOD, 51 | Clonal | ND |
| 11 | 25/F | L axilla | L axillary LN | − | T1aN1M0 | II | − | − | CHASE, PD | DOD, 8 | UD | Clonal |
CTCL-γδ, primary cutaneous T-cell lymphoma with T-cell receptor gamma-delta phenotype; SPTCL, subcutaneous panniculitis-like T-cell lymphoma; L, left; R, right; LN, Lymph node; BM, bone marrow; HPS, hemophagocytic sydrome; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisolone; THP-COP, cyclophosphamide, pirarubicin, vincristine, prednisolone; MINE, mesna, ifosfamide, mitoxantrone, etoposide; ESHAP, etoposide, methylprednisolone, cytarabine, cisplatin; MEPP, mitoxantrone, etoposide, cisplatin and prednisolone, BACOP, bleomycin, adriamycin, cyclophosphamide, vincristine, prednisone; CHASE, cyclophosphamide, cytosine arabinoside, etoposide, and dexamethasone; RT, radiotherapy; CHOP-B, cytoxan, adriamycin, vincristine, prednisone, bleomycin; VP-16, etoposide; INF-γ, interferon-gamma; PUVA, Psoralen Ultra-Violet A; ACNU, nimustine hydrochloride; CPA, cyclophosphamide; UVB, ultraviolet B; MTX, methotrexate; PD, progressive disease; NC, no change; CR, complete response; AWD, alive with disease; DOD, died of disease, PCR; polymerase chain reaction, TCR; T-cell receptor, ND; not done, UD; undetected.