Subcutaneous panniculitis-like T-cell lymphoma in a 19 month-old boy: a case report [Potkožni T-stanični limfom nalik panikulitisu u 19-mjesečnog dječaka: prikaz slučaja]

Rajić, Ljubica and Bilić, Ernest and Femenić, Ranka and Meštrović, Daniel and Ilić, Ivana and Lasan-Trčić, Ružica and Dubravčić, Klara and Husar, Karmela and Kardum-Skelin, Ika and Tešović, Goran and Čulig, Zdravka and Konja, Josip (2010) Subcutaneous panniculitis-like T-cell lymphoma in a 19 month-old boy: a case report [Potkožni T-stanični limfom nalik panikulitisu u 19-mjesečnog dječaka: prikaz slučaja]. Collegium Antropologicum, 34 (2). pp. 679-682. ISSN 0350-6134

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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. Immunohystochemical 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.

Abstract in Croatian

Potkožni T-stanični limfom nalik panikulitisu (engl. subcutaneous panniculitis-like T-cell lymphoma, SPTCL) jest rijetki oblik T-staničnog limfoma fenotipa CD3+CD8+ kojeg karakteriziraju duboki kožni čvorići ili plakovi nalik panniculitisu, a koji nastaju kao rezultat infiltracije potkožnog masnog tkiva neoplastičnim limfocitima. U ovom radu prikazujemo slučaj 19-mjesečnog dječaka sa SPTCL koji je dijagnosticiran i uspješno liječen u našoj ustanovi. Bolest se isprva prezentirala visokom vručicom i bolnim eritematoznim čvorićima ispod pupka. Kasnije su se infiltrati pojavili na licu, nogama, rukama i leđima. U postavljanju dijagnoze najodlučniji je bio patohistološki nalaz biopsije kože koji je pokazao infiltraciju atipičnih, malenih do srednje velikih limfatičnih stanica u dubljim slojevima dermisa i potkožnom tkivu oko adipocita. Na imunohistokemijskoj analizi neoplastični limfociti su izražavali biljege CD2, CD3, CD5, CD7, CD8, Tia-1, granzim-B i perforin, a bili su negativni na CD20, CD34, TDT i CD56. Nije nađena infiltracija krvnih žila i epidermisa. U djeteta je započeto liječenje protokolom specifičnim za T-stanične limfome (EURO-LB 02) što je uskoro dovelo do brzog povlačenja svih općih i lokalnih simptoma. Liječenje je završeno prema planu, a dijete je danas, 24 mjeseca od početka terapije u kompletnoj remisiji bolesti.

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