Diffuse large B-cell lymphoma in patient after treatment of angioimmunoblastic T-cell lymphoma [Difuzni B velikostanični limfom kod pacijentice nakon terapije angioimunoblastičnog T limfoma]

Džeko Škugor, Nives and Perić, Zinaida and Vrhovac, Radovan and Radić-Krišto, Delfa and Kardum-Skelin, Ika and Jakšić, Branimir (2010) Diffuse large B-cell lymphoma in patient after treatment of angioimmunoblastic T-cell lymphoma [Difuzni B velikostanični limfom kod pacijentice nakon terapije angioimunoblastičnog T limfoma]. Collegium Antropologicum, 34 (1). pp. 241-245. ISSN 0350-6134

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Abstract

Relatively few cases of Epstein-Barr (EBV)-positive B-cell lymphomas arising in patients with angioimmunoblastic T-cell lymphoma (AITL) have been reported. We report a case of AITL in which diffuse large B-cell lymphoma arose 13 months after the initial diagnosis of AITL. In a 36-year-old female patient, evaluated for moderate leukocytosis, peripheral and abdominal lymphadenopathy AITL was diagnosed in March 2008, based on results of fine-needle aspiration cytology (FNAC) of the enlarged cervical and supraclavicular lymph nodes.The diagnosis was also confirmed by immunophenotyping and histopathology of the cervical lymph nodes. The patient initially recieved FED chemotherapy (fludarabine, cyclophosphamide, dexamethasone) followed by elective autologous hematopoietic stem cell transplantation. In April 2009 the patient was hospitalized because of fever, pancytopenia, hyperbilirubinemia and peripheral lymphadenopathy. The FNAC of the enlarged cervical lymph nodes was performed again, but this time the smears were composed of polymorphous population of lymphocytes with the predomination of large cells, CD20+ on immunocytochemical stains. The immunophenotyping confirmed a predomination of monoclonal mature B-cells. Patient had high number of EBV DNA copies in plasma and serologic testing revealed increased titers of EBV VCA IgG and EBV EBNA IgG. CHOP-R chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) was then administered, resulting in good partial response of the disease. Reduced intensity allogeneic stem cell transplantation performed thereafter, resulted in complete remission of the disease. AITL is a rare lymphoproliferative disorder in which the neoplastic T-cells represent the minority of the lymph node cell population and almost all cases harbor EBV-infected B-cells. Various authors postulated that immunodeficiency in AITL patients together with immunosupresive effects of cytotoxic drugs, may be responsible for EBV-induced proliferation of latently or newely EBV-infected B-cells with eventual clonal selection and progression to aggressive B-cell lymphoma.

Abstract in Croatian

Relativno malo slučajeva Epstein-Barr virus (EBV) pozitivnih B staničnih limfoma nastalih kod pacijenata s angioimunoblastičnim T limfomom (AITL) je objavljeno. Opisujemo slučaj AITL-a kod kojeg je difuzni B velikostanični limfom nastao 13 mjeseci nakon prvotne dijagnoze AITL-a. Kod 36-godišnje pacijentice s umjerenom leukocitozom, perifernom i abdominalnom limfadenopatijom u ožujku 2008. godine dijagnosticiran je AITL nakon citološke punkcije povećanih cervikalnih i supraklavikularnih limfnih čvorova. Dijagnoza je potvrđena imunofenotipizacijom i biopsijom cervikalnih limfnih čvorova. Pacijentica je liječena kemoterapijom po shemi FED (fludarabin, ciklofosfamid, deksametazon) te joj je učinjena elektivna transplantacija autolognih krvotvornih matičnih stanica. U travnju 2009. godine pacijentica je hospitalizirana zbog vrućice, pancitopenije, hiperbilirubinemije i periferne limfadenopatije. Citološka punkcija povećanih cervikalnih limfnih čvorova je ponovo urađena, ali ovaj put u razmazima je nađena polimorfna populacija limfocita s predominacijom velikih stanica, imunocitokemijski CD20+. Imunofenotipizacija je potvrdila predominaciju monoklonalnih zrelih B stanica. Razina EBV DNA u plazmi bila je vrlo visoka, a serološkim je testiranjem dokazan povišen titar EBV VCA IgG i EBV EBNA IgG. Pacijentica je liječena imunokemoterapijom CHOP-R (ciklofosfamid, doksorubicin, vinkristin, prednizon i rituksimab) na koju je postignut vrlo dobar odgovor, a transplantacijom alogenih krvotvornih matičnih stanica nakon kondicioniranja smanjenog intenziteta postignuta je kompletna remisija. AITL je rijedak limfoproliferativni poremećaj kod kojeg neoplastične T stanice čine manjinu populacije limfnog čvora i u skoro svim slučajevima postoje EBV-om inficirane B stanice. Različiti autori pretpostavljaju da imunodeficijencija kod pacijenata s AITL-om zajedno s imunosupresivnim učinkom citotoksičnih lijekova bi mogla biti odgovorna za EBV-om induciranu proliferaciju latentno ili novoinficiranih B stanica uz eventualnu klonalnu selekciju i progresiju u agresivni B-stanični limfom.

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