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.
|Νέος χρήστης; Εγγραφή εδώ!|
|Υπενθύμιση στοιχείων εισόδου|
|Πληροφορίες για τις επιπλέον δυνατότητες των μελών|
Προμηθέας: Tαυτοποίηση και Aποκατάσταση θυμάτων βασανιστηρίων στην Ελλάδα
"Τα Οικονομικά του Καπνίσματος στην Ελλάδα"
«Τα Μετέωρα Βήματα του ΕΣΥ» Τριάντα χρόνια Εθνικού Συστήματος Υγείας