Cutaneous T-cell lymphoma;
Sezary syndrome
Α 61 year-old woman presented to the Outpatient Department with
generalized skin rash, alopekia, nail dystrophy and intense pruritus.
Two years ago the patient had developed eczematous skin rash;
there were skin patches of varying size, involving mainly the
unexposed areas of the body, and were accompanied by itching.
A tentative diagnosis of chronic eczematous dermatitis had been
made but the lesions had not responded to standard topical therapy.
A skin biopsy had been performed which had shown lymphoid infiltration,
mainly perivascular, without microabscesses and large atypical
lymphocytes. Immunophenotyping had revealed predominance of CD4+
helper cells, with absent CD7 and Leu-8 expression. The diagnosis
of “large plaque parapsoriasis” was made and the appropriate treatment
resulted in the improvement of skin lesions and itching. One and
a half years later the skin lesions and the pruritus recurred,
and the patient was admitted to our Department. On physical examination
there were scaly macules, and erythematous brown patches and plaques
with serpiginous borders in the whole body (fig.
1). There also were superficial atrophic scars, alopekia and
nail dystrophy. Cervical and supraclavicular microlymphadenopathy
(smaller than 0.5 cm in diameter, painless and mobile), and mild,
non-tender splenomegaly (2 cm below costal margin) were also present.
The hematology profile was as follows: Hb 14.2 g/dL, Ht 43%, WBC
6.7x109/L (differential count:
neutrophils 51%, lymphocytes 43%, monocytes 3%, eosinophils 1%
and atypical lymphocytes 2%) and platelets 198x109/L.
Peripheral blood smear showed atypical lymphocytes as shown in
figure
2. Serum biochemical tests revealed only a mild hypergammaglobulinemia
without a monoclonal component. Computed tomography scans of the
chest, abdomen and pelvis were normal. Bone marrow biopsy was
also negative for lymphocytic infiltration. Lymph node histology
revealed reactive changes. Skin biopsy revealed a moderately dense,
band-like, infiltrate of numerous, small to intermediate-size
lymphocytes in the superficial papillary dermis with epidermal
infiltration and formation of microabscesses. Immunophenotyping
comfirmed the origin of these cells: CD2+, CD3+, CD4+, CD5+, CD1-,
CD8-, CD25-. Total skin electron beam therapy and interferon administration
resulted in a significant improvement of skin lesions and of patient’s
quality of life.
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