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Blood, Vol. 95 No. 12 (June 15), 2000:
pp. 3653-3661
PLENARY PAPER
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
Marcel W. Bekkenk,
Françoise A. M. J. Geelen,
Pieter C. van Voorst Vader,
F. Heule,
Marie-Louise Geerts,
Willem A. van Vloten,
Chris J. L. M. Meijer, and
Rein Willemze
From the Department of Dermatology, Leiden University Medical
Center, Leiden; the Departments of Pathology and Dermatology, Free
University Hospital, Amsterdam; the Department of Dermatology,
University Hospital Groningen; the Department of Dermatology,
University Hospital Rotterdam; the Department of Dermatology,
University Hospital Gent; and the Department of Dermatology, University
Hospital Utrecht, The Netherlands.
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.
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