Guidelines for the Management of Haematological Malignancies

14 CUTANEOUS LYMPHOMA
The WHO classification of cutaneous T cell lymphomas
There are three main groups in decreasing order of prevalence:-
1. Mycosis fungoides and Sezary syndrome
Rare variants: Pagetoid reticulosis ( a verrucous acral variant)
MF-associated follicular mucinosis
Granulomatous slack skin disease
2. Primary cutaneous CD-30 positive T-cell lympho-proliferative disorders
Variants: Lymphomatoid papulosis (types A and B)
Primary cutaneous Anaplastic Large Cell Lymphoma
Borderline types
3. Other rare T cell lymphomas presenting in the skin
a. Subcutaneous panniculitis-like T-cell lymphoma
b. Peripheral T-cell lymphoma unspecified - an aggressive lymphoma which always
requires systemic therapy
Peripheral T-cell Lymphoma involving the skin at presentation
• The skin and sub-cutaneous tissue is commonly involved by systemic T-cell lymphomas.
• All of these lymphomas have an aggressive course and poor prognosis, even if they appear
localised to the skin at presentation.
• All of these tumours are treated using combination chemotherapy
• These patients must be referred urgently to a haemato-oncology MDT (Leeds, Hull, Mid-
Yorks, Bradford and Airedale, York & Harrogate).
• Peripheral T cell lymphoma should not be confused with CD30+ T-cell lymphoproliferative
disorders (see below).
Cutaneous CD30 positive lymphoproliferative disorders
Diagnostic Criteria
Typical Type A lymphomatoid papulosis
1. A wedge shaped lesion with a peripheral zone which contains a mixed inflammatory infiltrate
and a central zone containing atypical large lymphoid cells. In the central zone there may be
necrosis or ulceration.
2. The characteristic cells are large with highly anaplastic or multilobated nuclei. They have a Tcell
phenotype with strong CD30 expression. ALK1 is negative.
3. The definitive diagnosis requires a history of self-healing lesions.
Variants
1. CD30 + primary cutaneous anaplastic large cell lymphoma. The cells are the same as in
lymphomatoid papulosis but in the context of a progressive non-healing lesion. The presence
of ALK1 almost certainly implies that the tumour is a systemic anaplastic lymphoma, which
may be otherwise identical, and these markers must be shown to be negative for a diagnosis
of primary cutaneous T-cell lymphoma to be made. A final diagnosis can only be made after
staging investigations.
Patients with anaplastic lymphoma must be referred to a Haemato-oncology Unit URGENTLY
2. Type B lymphomatoid papulosis. This consists of small cells, similar to mycosis fungoides and
CD30 negative. Diagnosis only possible if typical history available.
Guidelines for the Management of Haematological Malignancies
14. CUTANEOUS LYMPHOMA
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