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Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: Report of the Dutch Cutaneous Lymphoma Group
Background: There is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). Radiotherapy (RT) and methotrexate (MTX) are the current treatment options, but their efficacy is unknown. Recently, targeted therapies showed promising results in C-ALCL, and may therefore be an attractive first choice of treatment.
Objectives: To assess the efficacy of conventional treatment strategies for patients with multifocal C-ALCL, and to define which patients may require novel targeted therapies.
Methods: In this multicenter study, treatment was evaluated in patients initially presenting (n=24) or relapsing with multifocal C-ALCL (n=17; 23 relapses). Distinction was made between cases with ≤ 5 (n=36) and >5 lesions (n=11).
Results: Treatments most commonly used were radiotherapy (n=21), systemic chemotherapy (n=9) and low-dose methotrexate (MTX; n=7) with complete response rates of 100%, 78% and 43%,...
Show moreBackground: There is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). Radiotherapy (RT) and methotrexate (MTX) are the current treatment options, but their efficacy is unknown. Recently, targeted therapies showed promising results in C-ALCL, and may therefore be an attractive first choice of treatment.
Objectives: To assess the efficacy of conventional treatment strategies for patients with multifocal C-ALCL, and to define which patients may require novel targeted therapies.
Methods: In this multicenter study, treatment was evaluated in patients initially presenting (n=24) or relapsing with multifocal C-ALCL (n=17; 23 relapses). Distinction was made between cases with ≤ 5 (n=36) and >5 lesions (n=11).
Results: Treatments most commonly used were radiotherapy (n=21), systemic chemotherapy (n=9) and low-dose methotrexate (MTX; n=7) with complete response rates of 100%, 78% and 43%, respectively, and an overall response rate of 100%, 100%, and 57%, respectively. Four patients showed a complete spontaneous regression. Sixteen of 24 patients (67%) first presenting with multifocal C-ALCL relapsed, including all five patients initially treated with CHOP. Compared with patients presenting with 2-5 skin lesions, patients presenting with >5 lesions had a higher chance of developing extracutaneous relapse (56 vs 20%) and more often died of lymphoma (44% vs 7%).
Conclusion: Patients with ≤5 lesions should be treated with low-dose RT (2x4 Gy). Maintenance low-dose MTX (20 mg/week) is a suitable option in patients with >5 lesions. Targeted therapies may be considered in rare patients refractory to MTX or patients developing extracutaneous disease.
Show less- All authors
- Melchers, R.C.; Willemze, R.; Bekkenk, M.W.; Haas, E.R.M. de; Horvath, B.; Rossum, M.M. van; Sanders, C.J.G.; Veraart, J.C.J.M.; Vermeer, M.H.; Quint, K.D.
- Date
- 2018-03-01