Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Lymph node biopsy if there is palpable adenopathy.
CT scans of the chest, abdomen and pelvis are indicated in
patients with non-mycosis fungoides CTCL variants, stage
IIA/B/III/IV mycosis fungoides and Sezary syndrome.
Scanning is not required in patients with stage IA/IB disease
or lymphomatoid papulosis.
Prognostic Factors / Index
Prognosis in mycosis fungoides is related to age at presentation
(worse if >60 years), clinical stage and elevated LDH. Patients
with limited stage disease may have a normal life expectancy, but
those with advanced disease have a poor prognosis, especially if
there is extra-cutaneous dissemination. Folliculotropic MF is less
accessible to skin-targeted therapies because of deep dermal
infiltration and disease-specific 5 year survival is approximately
70-80% which is worse than classical MF. The median survival in
Sézary syndrome is 32 months from diagnosis with an overall
survival of 10-20% at 5 years.
Potential Pitfalls
a. Diagnosis of cutaneous T cell lymphoma may be difficult
and differential diagnosis includes a number of other entities
including benign conditions such as small and large plaque
parapsoriasis, eczematous disorders and erythroderma
secondary to drug reactions, psoriasis or eczema .
b. Traditional anti-lymphoma regimens of combination
chemotherapy are not effective.
Treatment
■ Patients should be jointly managed by an
oncologist/haematologist and a dermatologist.
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Skin-directed therapy (phototherapy, topical therapy or
superficial radiotherapy) is appropriate for patients with early
stage mycosis fungoides (stages IA-IIA) with the choice of
therapy dependent on the extent of cutaneous disease and
plaque thickness. Agents used include topical high-intensity
steroids and bexarotene gel.
Combined PUVA and α-interferon therapy can be effective
for patients with resistant early-stage disease (stage IB-IIA).
Systemic therapy is required for patients with stage IIB
or higher mycosis fungoides with interferon or one of the
newer agents described below.
Bexarotene is an oral retinoid with an overall response rate
of about 45% as monotherapy which is increased when
combined with interferon and/or PUVA. Bexarotene causes
central hypothyroidism and hypertryglyceridaemia which
must be monitored and treated appropriately
■ Gemcitabine monotherapy is also active in CTCL, using 1000
mg/m2 on day 1,8 +/-15 resulting in an ORR of 68% in
heavily pre-treated patients.
■ Campath IH given iv or sc three times a week for up to 12
weeks in patients with heavily pre-treated, advanced disease
resulted in an ORR of 38%-55%, but associated with a high
incidence of infections
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