Primary and secondary cutaneous CD301 lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

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 calcu- lated 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 con- firm the favorable prognoses of these primary cutaneous CD301 lymphoprolifer- ative disorders and underscore that LyP and primary cutaneous CD301 lympho- mas are closely related conditions. They also indicate that CD30 1 LTCL on the skin and in 1 draining lymph node station has a good prognosis similar to that for primary cutaneous CD301 LTCL without concurrent lymph node involvement. Mul- tiagent chemotherapy is only indicated for patients with full-blown or developing extracutaneous disease; it is never or rarely indicated for patients with skin- limited CD301 lymphomas. (Blood. 2000; 95:3653-3661)