Peripheral (cutaneous) T-cell lymphoma, unspecified (according to WHO 2008 classification for lymphoid neoplasms)
Journal Title: Αρχεία Ελληνικής Ιατρικής - Year 2009, Vol 26, Issue 6
Abstract
A 67-year-old man was referred to our center for the evaluation of anemia and the presence of a 2-month history of multiple erythematous patches associated with few papulonodular and blistering lesions located mainly on the trunk. His medical history included type 1 diabetes mellitus and hypertension. His physical examination revealed erythematous arciform, annular patches and disseminated nodules, most of which had an ulcerated, hemorrhagic surface, on trunk and axillary regions. There was a mild hypertension (145/95 mmHg) but there was no organomegaly or lymphadenopathy. His full blood count showed a mild anemia (Hb 11.2 g/dL, Ht 34.8%, MCV 89 fL, MCH 30.6 pg) with normal white blood cell and platelet counts. In the evaluation of peripheral blood smears, the presence of a small number (<1%) of atypical medium/large pleomorphic lymphocytes was observed. There was an increased erythrocyte sedimentation rate (88 mm/h) and elevated serum β2-microglobulin (2.4 mg/mL). Serological tests for Epstein-Barr virus and cytomegalovirus suggested previous infection, whereas serology for hepatitis B and C viruses and human herpesvirus (HHV)-6, HHV-7, and HHV-8 produced negative findings. The bone marrow trephine biopsy and the marrow aspiration revealed no infiltration by clonal lymphocytes. Histology from a nodule showed a massive infiltrate of large lymphocytes with a discrete number of blastic lymphoid cells throughout the entire dermis, with diffuse infiltration of the epidermis. Immunohistochemical studies on paraffin-embedded tissue demonstrated that the malignant cells had an α/β T-helper phenotype (CD3+, CD4+, βF1+, T-cell receptor [TCR]-δ1–, CD56–); most of the neoplastic cells were proliferating (mindbomb homolog 1+) and expressed the CD30 antigen. TCR-γ gene rearrangement analysis of a biopsy specimen from lesional skin, using a polymerase chain reaction technique showed a monoclonal gene rearrangement. The diagnosis was established and specific therapy started. Initial treatment produced only partial regression of the skin lesions within a 6-month period.
Authors and Affiliations
A. SARANTOPOULOS, J. ASIMAKOPOULOS, E. PAPAKOSTAS, T. CHATZILEONIDA, M. MICHAEL, O. KAMPOULOPOULOU, N. VINIOU
Keywords
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