Clinical characteristics of the 15 patients
Patient . | Sex/age . | Symptoms . | No. Sézary cells mm3 (%) . | Delay of diagnosis . | Previous treatment . | Current treatment . |
---|---|---|---|---|---|---|
N | F/76 | Erythroderma, PPK, nail dystrophy | 400 (6) | Few wk | 0 | Chlormetine |
Bro | M/61 | Pruritus, erythroderma | 1 750 (25) | 10 mo | Chlormetine, IFN, BCNU, MTX, MTX + pUVA | ECP: 2 per wk then 1 per 15 d then 2 per wk every 3 wk |
T | F/79 | Pruritus, purpuric erythema | (24) | 3 mo | pUVA, UVA + UVB, chlormetine, ECP + MTX | IFN + CS |
Ve | F/69 | Pruritus, discreet erythema, then erythroderma, lymph nodes | 1 500 (12) | 4 y | Chlormetine | PUVA |
S | M/53 | Exfoliative erythroderma, PPK | 100 (1) | 18 mo | PUVA | IFN |
G | F/46 | Pruritus, exfoliative erythroderma, PPK | 1 200 (9) | 8 mo | PUVA, chlormetine, IFN, MTX, ECP | ECP (intensive) + CS |
V | M/88 | Exfoliative erythroderma, PPK, inguinal lymphadenopathy | 194 (2) | 4 mo | Chlormetine, BCNU | ECP (intensive) then CHOP 6 cures because of histologic transformation |
Sa | M/62 | Pruritus, erythroderma | 1 000 (9) | 18 mo | PUVA | ECP (classical) alone then ECP + IFN |
Vd | M/65 | Pruritus, exfoliative erythroderma, PPK, nail dystrophy, lymphadenopathy | 8 000 (43) | 3 y | 0 | ECP (intensive) then MTX |
Bra | M/70 | Pruritus, reticular erythema | (4) | — | Chlormetine, CS, pUVA then pUVA+, chloraminophen, IFN alone then + cytapheresis and acitretin | ECP (classical) + acitretin |
Ciz | M/62 | Exfoliative erythroderma, PPK | 2 200 (22) | 1 y | CS, chlormetine, chloraminophen, IFN, MTX | ECP (classical) |
Sy | F/75 | Pruritus, xerosis, annular erythematous lesions, then exfoliative erythroderma | 26 000 (88) | 1 y | pUVA | ECP (intensive) |
Sv | M/60 | Exfoliative erythroderma, pruritus, PPK, nail dystrophy | (56) | 6 y | Cytapheresis, chlormetine, pUVA, IFN | ECP (classical) then 2 per d for 15 d then 1 per d for 15 d, ECP + IFN |
R | M/59 | Pruritus, erythroderma, axillary lymphadenopathy | 2 000 (25) | 3-4 y | PUVA, chlormetine | ECP (intensive then classical) |
D | M/53 | Exfoliative erythroderma, pruritus, PPK, fever, systemic signs (pulmonary, hepatic, intestinal, neurologic) | 1 100 (14) | Few mo | 0 | CHOP > 6 cures |
Patient . | Sex/age . | Symptoms . | No. Sézary cells mm3 (%) . | Delay of diagnosis . | Previous treatment . | Current treatment . |
---|---|---|---|---|---|---|
N | F/76 | Erythroderma, PPK, nail dystrophy | 400 (6) | Few wk | 0 | Chlormetine |
Bro | M/61 | Pruritus, erythroderma | 1 750 (25) | 10 mo | Chlormetine, IFN, BCNU, MTX, MTX + pUVA | ECP: 2 per wk then 1 per 15 d then 2 per wk every 3 wk |
T | F/79 | Pruritus, purpuric erythema | (24) | 3 mo | pUVA, UVA + UVB, chlormetine, ECP + MTX | IFN + CS |
Ve | F/69 | Pruritus, discreet erythema, then erythroderma, lymph nodes | 1 500 (12) | 4 y | Chlormetine | PUVA |
S | M/53 | Exfoliative erythroderma, PPK | 100 (1) | 18 mo | PUVA | IFN |
G | F/46 | Pruritus, exfoliative erythroderma, PPK | 1 200 (9) | 8 mo | PUVA, chlormetine, IFN, MTX, ECP | ECP (intensive) + CS |
V | M/88 | Exfoliative erythroderma, PPK, inguinal lymphadenopathy | 194 (2) | 4 mo | Chlormetine, BCNU | ECP (intensive) then CHOP 6 cures because of histologic transformation |
Sa | M/62 | Pruritus, erythroderma | 1 000 (9) | 18 mo | PUVA | ECP (classical) alone then ECP + IFN |
Vd | M/65 | Pruritus, exfoliative erythroderma, PPK, nail dystrophy, lymphadenopathy | 8 000 (43) | 3 y | 0 | ECP (intensive) then MTX |
Bra | M/70 | Pruritus, reticular erythema | (4) | — | Chlormetine, CS, pUVA then pUVA+, chloraminophen, IFN alone then + cytapheresis and acitretin | ECP (classical) + acitretin |
Ciz | M/62 | Exfoliative erythroderma, PPK | 2 200 (22) | 1 y | CS, chlormetine, chloraminophen, IFN, MTX | ECP (classical) |
Sy | F/75 | Pruritus, xerosis, annular erythematous lesions, then exfoliative erythroderma | 26 000 (88) | 1 y | pUVA | ECP (intensive) |
Sv | M/60 | Exfoliative erythroderma, pruritus, PPK, nail dystrophy | (56) | 6 y | Cytapheresis, chlormetine, pUVA, IFN | ECP (classical) then 2 per d for 15 d then 1 per d for 15 d, ECP + IFN |
R | M/59 | Pruritus, erythroderma, axillary lymphadenopathy | 2 000 (25) | 3-4 y | PUVA, chlormetine | ECP (intensive then classical) |
D | M/53 | Exfoliative erythroderma, pruritus, PPK, fever, systemic signs (pulmonary, hepatic, intestinal, neurologic) | 1 100 (14) | Few mo | 0 | CHOP > 6 cures |