Main clinical, biological characteristics, and treatments in 70 patients with subcutaneous panniculitis-like T-cell lymphoma in the study cohort
Patients . | n = 70 (%) . |
---|---|
Ethnicity | |
Europe | 37/63 (59) |
Asia* | 9/63 (14) |
North Africa | 7/63 (11) |
Sub-Saharan Africa | 4/63 (6) |
Caribbean Islands | 3/63 (5) |
Polynesia | 3/63 (5) |
History of autoimmune disease | 25/68 (37) |
Lupus erythematosus | 13/68 (19) |
Rheumatoid arthritis | 4/68 (6) |
Antiphospholipids syndrome | 4/68 (6) |
Overlap syndrome | 2/68 (3) |
Autoimmune cytopenia | 2/68 (3) |
Autoimmune thyroiditis | 2/68 (3) |
Autoimmune cytopenia | 2/68 (3) |
Multiple sclerosis | 1/68 (2) |
Alopecia areata | 1/68 (2) |
Clinical abnormalities | |
Lesions size | |
1-5 cm | 16/38 (42) |
5-10 cm | 12/38 (32) |
>10 cm | 9/38 (24) |
Morphology of the lesions | |
Nodules | 53/61 (87) |
Plaques | 16/61 (26) |
Ulceration | 6/61 (10) |
Number of lesions | |
Single | 5/68 (7) |
Multiple | 63/68 (93) |
Localization of the lesions | |
Upper limb | 36/68 (53) |
Lower limb | 42/68 (62) |
Trunk | 37/68 (54) |
Head | 15/68 (22) |
B symptoms | 46/67 (69) |
Fever | 36/67 (54) |
Weight loss | 19/67 (28) |
Asthenia | 36/67 (54) |
Lymphadenopathy | 21/66 (32) |
Splenomegaly | 11/65 (17) |
Hepatomegaly | 11/66 (17) |
Biological abnormalities | |
Hemophagocytic syndrome | 12/68 (18) |
Auto-antibodies | 32/52 (62) |
Anti-nuclear antibodies (titer > 1/80) | 30/52 (58) |
Treatments | |
Polychemotherapy† | 17/68 (25) |
Polychemotherapy as first-line treatment | 11/68 (16) |
Followed by an autologous stem cell transplantation | 4/68 (6) |
Followed by an allogeneic stem cell transplantation | 1/68 (2) |
Polychemotherapy as second-line treatment | 6/68 (9) |
Followed by an autologous stem cell transplantation | 1/68 (2) |
Followed by an allogeneic stem cell transplantation | 1/68 (2) |
Immunosuppressive drug as first-line treatment | 52/68 (77) |
Corticosteroids | 16/68 (24) |
Corticosteroids + low-dose methotrexate | 12/68 (18) |
Hydroxychloroquine | 8/68 (12) |
Low-dose methotrexate | 7/68 (10) |
Corticosteroids + hydroxychloroquine | 2/68 (3) |
α-interferon | 2/68 (3) |
Chloraminophene | 1/68 (2) |
Corticosteroids + cyclosporine | 1/68 (2) |
Patients . | n = 70 (%) . |
---|---|
Ethnicity | |
Europe | 37/63 (59) |
Asia* | 9/63 (14) |
North Africa | 7/63 (11) |
Sub-Saharan Africa | 4/63 (6) |
Caribbean Islands | 3/63 (5) |
Polynesia | 3/63 (5) |
History of autoimmune disease | 25/68 (37) |
Lupus erythematosus | 13/68 (19) |
Rheumatoid arthritis | 4/68 (6) |
Antiphospholipids syndrome | 4/68 (6) |
Overlap syndrome | 2/68 (3) |
Autoimmune cytopenia | 2/68 (3) |
Autoimmune thyroiditis | 2/68 (3) |
Autoimmune cytopenia | 2/68 (3) |
Multiple sclerosis | 1/68 (2) |
Alopecia areata | 1/68 (2) |
Clinical abnormalities | |
Lesions size | |
1-5 cm | 16/38 (42) |
5-10 cm | 12/38 (32) |
>10 cm | 9/38 (24) |
Morphology of the lesions | |
Nodules | 53/61 (87) |
Plaques | 16/61 (26) |
Ulceration | 6/61 (10) |
Number of lesions | |
Single | 5/68 (7) |
Multiple | 63/68 (93) |
Localization of the lesions | |
Upper limb | 36/68 (53) |
Lower limb | 42/68 (62) |
Trunk | 37/68 (54) |
Head | 15/68 (22) |
B symptoms | 46/67 (69) |
Fever | 36/67 (54) |
Weight loss | 19/67 (28) |
Asthenia | 36/67 (54) |
Lymphadenopathy | 21/66 (32) |
Splenomegaly | 11/65 (17) |
Hepatomegaly | 11/66 (17) |
Biological abnormalities | |
Hemophagocytic syndrome | 12/68 (18) |
Auto-antibodies | 32/52 (62) |
Anti-nuclear antibodies (titer > 1/80) | 30/52 (58) |
Treatments | |
Polychemotherapy† | 17/68 (25) |
Polychemotherapy as first-line treatment | 11/68 (16) |
Followed by an autologous stem cell transplantation | 4/68 (6) |
Followed by an allogeneic stem cell transplantation | 1/68 (2) |
Polychemotherapy as second-line treatment | 6/68 (9) |
Followed by an autologous stem cell transplantation | 1/68 (2) |
Followed by an allogeneic stem cell transplantation | 1/68 (2) |
Immunosuppressive drug as first-line treatment | 52/68 (77) |
Corticosteroids | 16/68 (24) |
Corticosteroids + low-dose methotrexate | 12/68 (18) |
Hydroxychloroquine | 8/68 (12) |
Low-dose methotrexate | 7/68 (10) |
Corticosteroids + hydroxychloroquine | 2/68 (3) |
α-interferon | 2/68 (3) |
Chloraminophene | 1/68 (2) |
Corticosteroids + cyclosporine | 1/68 (2) |
Including: Vietnam (n = 4), Cambodia (n = 2), Bangladesh (n = 1), China (n = 1), not precise (n = 1)
Including the following regimen: CHOP (cyclophosphamide, adriamycin, vincristine, prednisone); CHOEP (CHOP + etoposide); ESHAP (etoposide, methylprednisolone, cisplatin, cytarabine); doxorubicin and cyclophosphamide; gemcitabine and oxaliplatin