Preliminary communication
Subcutaneous Panniculitis-like T-cell Lymphoma in a 19 Month-old Boy: A Case Report
Ljubica Rajić
Ernest Bilić
Ranka Femenić
Daniel Meštrović
Ivana Ilić
Ružica Lasan-Trčić
Klara Dubravčić
Karmela Husar
Ika Kardum-Skelin
Goran Tešović
Zdravka Čulig
Josip Konja
Abstract
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma of CD3+CD8+ phenotype characterized by deep-seated skin nodules or plaques mimicking panniculitis, a result of neoplastic lymphocytes infiltrating the subcutaneous fatty tissue. We present a case of a 19-month year old boy with SPTCL diagnosed and successfully treated in our institution. Disease first presented with symptoms of high fever and painful erythematous nodule located below the umbilicus. Later on the infiltrates appeared on the face, legs, arms and the back of the body. As the most decisive in obtaining the diagnosis, skin biopsy showed atypical, small to medium-sized lymphatic cells infiltrating the deeper dermal layers as well as the subcutaneous adipous tissue surrounding the adipocytes. Imunohystochemical analysis showed neoplastic lymphocytes positive for CD2, CD3, CD5, CD7, CD8, Tia-1, granzyme B and perforine, and negative for CD20, CD34, TDT and CD56. No infiltration of blood vessels or epidermis was evident. Specific T-cell lymphomas protocol (EURO-LB 02) was then initiated which resulted with rapid regression of all general and local symptoms. The treatment was completed according to schedule and the child is now, 24 months after the initiation of the treatment, in complete remission.
Keywords
subcutaneous panniculitis-like T-cell lymphoma; children; hemophagocytic syndrome; chemotherapy
Hrčak ID:
56527
URI
Publication date:
30.6.2010.
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