Primary cutaneous large-cell lymphoma: analysis of 49 patients included in the LNH87 prospective trial of polychemotherapy for high-grade lymphomas

Abstract

The objectives of this study were to evaluate the outcome after polychemotherapy for patients with primary cutaneous largecell lymphomas (PCLL) and to validate the recently proposed immunohistologic classification of cutaneous lymphomas. Among 140 patients with positive skin biopsies included in the LNH87 protocol (for treatment of aggressive lymphomas), 49 patients met the criteria of PCLL. Characteristics were: sex ratio M/F, 2.3; age 18 to 83 years (median, 52), peripheral lymph nodes, n = 22; diffuse disease, n = 12; median tumor size, 4.5 cm; elevated lactate dehydrogenase, n = 9; ECOG: 0/1, n = 49. Histology was: follicular center B cell, n = 23; B-lymphoblastic, n = 1; anaplastic large-cell lymphoma, n = 14 (T cell phenotype n = 8); CD30 ؊ T cell lymphoma, n = 11. All patients received polychemotherapy: under 70 years, ACVBP (three to four cycles and consolidation for 6 months) n = 25; mBACOD (eight cycles) n = 16; over 70 years, C(T)VP (six cycles) n = 8. Radiation therapy was not included in the protocol. With a median follow-up of 5 years, 24/49 patients had relapsed, with 20 skin relapses. Event-free (EFS) and overall survival (OS) at 5 years were, respectively, 50 and 77%. Significant adverse prognostic factors were: histology (CD30 ؊ T cell lymphoma) and diffuse cutaneous disease (Ͼ Ͼ Ͼ10% of skin). The presence of nodal involvement was only significant for EFS. When compared to 140 non-cutaneous lymphoma patients included in the same trial and fully matched for the main clinical characteristics, OS was similar. In conclusion, PCLL behaves like other localized B or T cell extranodal lymphomas with the same prognostic factors (LDH, ECOG, age) except for CD30 ؉ PCLL which have a very good prognosis.

Bcl-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas

“…on May 10, 2018. by guest www.bloodjournal.org From Although primary cutaneous lymphomas were theoretically defined in the EORTC classification by the absence of extracutaneous progression within 6 months after diagnosis, we adopted in the present study the pragmatic, widely used definition based on a complete negative staging at diagnosis. 5,7,10,11,13,28,36 This definition was recently recommended for 2 major reasons: (1) aggressive lymphomas that arise in the skin may show dissemination before a period of 6 months; (2) patients need to be treated at presentation, thus a clear-cut diagnosis must be established immediately, not 6 months afterward. 5 In our series, 4 of 80 patients had extracutaneous progression (restricted to lymph nodes) within 6 months after diagnosis.…”

Non-Hodgkin's Lymphomas

“…374 In patients presenting with PCFCL, multiagent chemotherapy and RT were equally effective for multifocal skin lesions. [375][376][377] Rituximab has been effective as a first-line treatment option for patients with indolent CBCLs with multiple lesions for which local therapy is not effective. [378][379][380][381][382] In a series of 16 patients with PCBCL, 14 patients (87.5%) experienced complete remission, with 35% of these patients experiencing relapse between 6 and 37 months.…”

Primary Cutaneous Lymphomas and Interferon Treatment

“…Remissions with cytotoxic chemotherapy, occurring rapidly even after low-dose methotrexate or etoposide, are disappointingly short (months) in refractory cases, although retreatment is often successful. Anecdotal responses to imiquimod, bexarotene, retinoids, interferon, and thalidomide have been reported [26,27]. Second sub-type is Lymphomatoid papulosis (LyP).…”