Primary and secondary cutaneous CD30(+) 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

To evaluate our diagnostic and therapeutic guidelines, clinical and long-te rm follow-up data of 219 patients with primary or secondary cutaneous CD30( +) lympho proliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with p rimary cutaneous CD30(+) large T-cell lymphoma (LTCL; group 2), 11 patients with CD30(+) LTCL and skin and regional lymph node involve ment (group 3), and 11 patients with secondary cutaneous CD30(+) LTCL (group 4), Patients with LyP often did not receive any specific treatment, whereas most patient s with primary cutaneous CD30(+) LTCL were treated with radiotherapy or exc ision, 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 gro up 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% (gro up 3), and 24% (group 4), respectively. The results confirm the favorable p rognoses of these primary cutaneous CD30(+) lymphoproliferative disorders a nd 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 pri mary cutaneous CD30(+) LTCL without concurrent lymph node involvement. Mult iagent chemotherapy is only indicated for patients with full-blown or devel oping extracutaneous disease; it is never or rarely indicated for patients with skin-limited CD30(+) lymphomas. (C) 2000 by The American Society of He matology.