Primary cutaneous lymphomas: diagnosis and treatment

Abstract

Primary cutaneous lymphomas (CLs) are a heterogeneous group of lymphoproliferative neoplasms, with lymphatic proliferation limited to the skin with no involvement of lymph nodes, bone marrow or viscera at the diagnosis. Cutaneous lymphomas originate from mature T-lymphocytes (65% of all cases), mature B-lymphocytes (25%) or NK cells. Histopathological evaluation including immunophenotyping of the skin biopsy specimen is the basis of the diagnosis, which must be complemented with a precise staging of the disease and identification of prognostic factors, to allow for the choice of the best treatment method as well as for the evaluation of the treatment results.

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“…Pierwotne CTCL definiuje się jako te, które przez minimum 6 miesięcy od rozpoznania są ograniczone wyłącznie do skóry, bez zajęcia wę-złów chłonnych, szpiku kostnego i narządów wewnętrznych. Stanowią one ok. 65% wszystkich chło-niaków pierwotnie skórnych [1,2].…”

“…The incidence of MF is estimated at approximately 0.5-1 case/100,000 population, with a peak age of onset at 55-60 years, and the disease affects men twice as often as women [1,2,[4][5][6]. It is characterized by a chronic progressive course comprising several stages (initial premycotic; stadium premycoticum), infiltrative (stadium infiltrativum) and tumorous (stadium tumorosum) which may overlap, though not all of them occur in a succession in every patient.…”

“…At early stages, diagnosis is made on the basis of clinical manifestations, however it should preferably be confirmed by a histopathological examination of biopsy material taken from the skin or, if possible, from a lymph node or another disease-affected organ, followed by immunophenotyping [1]. Cellular immunophenotyping to determine the set of surface antigens should be performed in every case, either by immunohistochemical examination of a tissue fragment or flow cytometry of a cell suspension.…”

“…It is characterized by a chronic progressive course comprising several stages (initial premycotic; stadium premycoticum), infiltrative (stadium infiltrativum) and tumorous (stadium tumorosum) which may overlap, though not all of them occur in a succession in every patient. Furthermore, the majority of them do not usually progress to advanced stages [1,4,7].…”

“…Primary CTCLs are defined as those which, for a minimum of 6 months after diagnosis, are limited to the skin, without any involvement of the lymph nodes, bone marrow or internal organs. Primary CTCLs account for approximately 65% of all primary cutaneous lymphomas [1,2].…”