To evaluate our diagnostic and therapeutic guidelines, clinical and long-term follow-up data of 219 patients with primary or secondary cutaneous CD30+ lymphoproliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with primary cutaneous CD30+ large T-cell lymphoma (LTCL; group 2), 11 patients with CD30+ LTCL and skin and regional lymph node involvement (group 3), and 11 patients with secondary cutaneous CD30+ LTCL (group 4). Patients with LyP often did not receive any specific treatment, whereas most patients with primary cutaneous CD30+ LTCL were treated with radiotherapy or excision. All patients with skin-limited disease from groups 1 and 2 who were treated with multiagent chemotherapy had 1 or more skin relapses. The calculated risk for systemic disease within 10 years of diagnosis was 4% for group 1, 16% for group 2, and 20% for group 3 (after initial therapy). Disease-related 5-year-survival rates were 100% (group 1), 96% (group 2), 91% (group 3), and 24% (group 4), respectively. The results confirm the favorable prognoses of these primary cutaneous CD30+ lymphoproliferative disorders and underscore that LyP and primary cutaneous CD30+ lymphomas are closely related conditions. They also indicate that CD30+ LTCL on the skin and in 1 draining lymph node station has a good prognosis similar to that for primary cutaneous CD30+ LTCL without concurrent lymph node involvement. Multiagent chemotherapy is only indicated for patients with full-blown or developing extracutaneous disease; it is never or rarely indicated for patients with skin-limited CD30+ lymphomas.

In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30 ؊ peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30 ؊ large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small-or mediumsized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30 ؊ large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small-or mediumsized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3 ؉ CD4 ؉ CD8 ؊ phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4 ؉ or CD8 ؉ phenotype. The only exception was a group of primary cutaneous small-or medium-sized pleomorphic CTCLs with a CD3 ؉ CD4 ؉ CD8 ؊ phenotype and presenting with localized skin lesions. (Blood.

With site, morphology, and number of tumors taken into account, guidelines for the management of PCLBCL are presented.