title: 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.
abstract: 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. (Blood. 2000;95:3653-3661)
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: M W
authors.initials: MW
authors.lastname: Bekkenk
authors.forename: F A
authors.initials: FA
authors.lastname: Geelen
authors.forename: P C
authors.initials: PC
authors.lastname: van Voorst Vader
authors.forename: F
authors.initials: F
authors.lastname: Heule
authors.forename: M L
authors.initials: ML
authors.lastname: Geerts
authors.forename: W A
authors.initials: WA
authors.lastname: van Vloten
authors.forename: C J
authors.initials: CJ
authors.lastname: Meijer
authors.forename: R
authors.initials: R
authors.lastname: Willemze
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D009426:Netherlands; D017410:Practice Guidelines as Topic; D012867:Skin / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D015996:Survival Rate; D013997:Time Factors
chemical_list: D015703:Antigens, CD; D017730:Ki-1 Antigen