R C Melchers1, R Willemze1, M W Bekkenk2, E R M de Haas3, B Horvath4, M M van Rossum5, C J G Sanders6, J C J M Veraart7, M H Vermeer1, K D Quint1. 1. Department of Dermatology, Leiden University Medical Center, the Netherlands. 2. Department of Dermatology, Academic Medical Center and Vrije University Medical Center, Amsterdam, the Netherlands. 3. Department of Dermatology, Erasmus Medical Center, Rotterdam, the Netherlands. 4. Department of Dermatology, University Medical Center of Groningen, the Netherlands. 5. Department of Dermatology, Radboud University Medical Center, Nijmegen, the Netherlands. 6. Department of Dermatology, University Medical Center Utrecht, the Netherlands. 7. Department of Dermatology, Maastricht University Medical Center, the Netherlands.
Abstract
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 multicentre study, treatment was evaluated in patients initially presenting (n = 24) or relapsing with multifocal C-ALCL (n = 17; 23 relapses). Distinction was made between patients with five or less lesions (n = 36) and more than five lesions (n = 11). RESULTS: Treatments most commonly used were RT (n = 21), systemic chemotherapy (n = 9) and low-dose 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 complete spontaneous regression. In total, 16 of 24 patients (67%) first presenting with multifocal C-ALCL relapsed, including all five patients initially treated with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone). Compared with patients presenting with two to five skin lesions, patients presenting with more than five lesions had a higher chance of developing extracutaneous relapse (56% vs. 20%) and more often died of lymphoma (44% vs. 7%). CONCLUSIONS: Patients with five or less lesions should be treated with low-dose RT (2 × 4 Gy). Maintenance low-dose MTX (20 mg weekly) is a suitable option in patients with more than five lesions. Targeted therapies may be considered in rare patients who are refractory to MTX or patients developing extracutaneous disease.
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 multicentre study, treatment was evaluated in patients initially presenting (n = 24) or relapsing with multifocal C-ALCL (n = 17; 23 relapses). Distinction was made between patients with five or less lesions (n = 36) and more than five lesions (n = 11). RESULTS: Treatments most commonly used were RT (n = 21), systemic chemotherapy (n = 9) and low-dose 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 complete spontaneous regression. In total, 16 of 24 patients (67%) first presenting with multifocal C-ALCL relapsed, including all five patients initially treated with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone). Compared with patients presenting with two to five skin lesions, patients presenting with more than five lesions had a higher chance of developing extracutaneous relapse (56% vs. 20%) and more often died of lymphoma (44% vs. 7%). CONCLUSIONS:Patients with five or less lesions should be treated with low-dose RT (2 × 4 Gy). Maintenance low-dose MTX (20 mg weekly) is a suitable option in patients with more than five lesions. Targeted therapies may be considered in rare patients who are refractory to MTX or patients developing extracutaneous disease.
Authors: Jong Bin Park; Myeong Hyeon Yang; Do Ik Kwon; Seol Hwa Seong; Ji Yun Jang; Kee Suck Suh; Min Soo Jang Journal: Acta Derm Venereol Date: 2020-02-29 Impact factor: 3.875
Authors: Federico Meconi; Roberto Secchi; Raffaele Palmieri; Sara Vaccarini; Vito Mario Rapisarda; Laura Giannì; Fabiana Esposito; Ida Provenzano; Daniela Nasso; Livio Pupo; Maria Cantonetti Journal: Case Rep Hematol Date: 2019-09-17
Authors: Antonello Sica; Paola Vitiello; Andrea Ronchi; Beniamino Casale; Armando Calogero; Evangelista Sagnelli; Gilca Costa Nachtigal; Teresa Troiani; Renato Franco; Giuseppe Argenziano; Elvira Moscarella; Caterina Sagnelli Journal: Int J Environ Res Public Health Date: 2020-01-29 Impact factor: 3.390