Guidelines for the Management of Haematological Malignancies

Notes:
1. The immunophenotypic investigation of T-cell lymphoma is being reviewed and a new protocol
is likely.
2. Clonality studies in the absence of the morphological immunophentypic and clinical features
are of no value in making a diagnosis of T-cell lymphoma and are potentially misleading
because of false positives.
3. In granulomatous slack skin disease, diffuse granulomata and lymphocytic infiltrate are seen
in the dermis. Macrophage mediated destruction of elastic tissue occurs which leads to the
development of pendulous folds of slack skin.
Mycosis Fungoides
Diagnosis requires:
1. The development of tumour nodules in the clinical background of established mycosis
fungoides
2. A cohesive population of large lymphoid cells within the tumour; in some cases CD30+
3. Cell cycle fraction in this population greater than 30%
4. Abnormal P53 expression.
Lymph Node Involvement
Involved lymph nodes in MF/Sezary show a range of changes histologically. Some units use a grading
system (category I to III after Clendenning and Rappaport) and in others molecular methods are being
evaluated. We recommend however that this system be adopted.
1. Dermatopathic reaction only: which may include scattered atypical lymphocytes or even small
clusters.
2. Focal or diffuse nodal replacement by tumour cells.
Sezary Syndrome
Sezary syndrome is characterized by erythroderma, lymphadenopathy, splenomegaly and leukaemia
(Greenberg, Cox et al. 1997; Wood and Greenberg 2003)
The diagnosis requires:
1 Generalised erythroderma
2 A skin biopsy showing infiltration by atypical T-cell as described for MF although the
histological appearances may be difficult to interpret and obscured by secondary infection
(Wood and Greenberg 2003)
3 Population of circulating T-cell with the phenotype CD4+,CD7- by flow cytometry. This
population must exceed 1x10 9 /l.
4 T-cell clonality as above
Notes:
1. Patients will be classified as primary or secondary according to the presence of preceding
mycosis fungoides
2. Although morphologically derived “counts” of Sezary cells are widely used in definitions of
Sezary this test is inaccurate and poorly reproducible (Wood and Greenberg 2003)and
therefore will not be performed.
3. The detection of circulating cells with the above phenotype is not a test for common type
mycosis fungoides and will not be performed in the absence of a diagnosis of erythroderma
and histological evidence of MF or lymphocytosis.
4. All patients with a diagnosis of Sezary syndrome should be referred to an appropriate
Haematology MDT.
Staging investigations
When the diagnosis of MF/Sezary is histologically verified, the following staging investigations are
recommended:
• FBC, ESR, differential WCC
Guidelines for the Management of Haematological Malignancies
14. CUTANEOUS LYMPHOMA
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