Extranodal marginal zone non Hodgkin's lymphoma of the lung: A ten-year experience

Abstract

Background/Aim. Bronchus-associated lymphoid tissue (BALT) lymphoma is a rare subtype of low grade marginal zone B cell lymphoma representing 10% of all MALT lymphomas. The purpose of this study was to analyze the outcome of this group of patients comparing prognostic parameters and therapy modalities. Methods. A total of eight patients with BALT lymphoma had diagnosed between January 1998 - April 2008 at the Institute of Hematology, Clinical Center of Serbia, Belgrade, and they were included in this retrospective analysis. Results. Male/female ratio was 2/6, the median age was 64 years (range 37-67 years). Six patients had nonspecific respiratory symptoms and all of them had B symptoms. The patients were seronegative for HIV, HCV and HBsAg. Three patients had Sjogren's syndrome, rheumatoid arthritis and pulmonary tuberculosis, respectively. Seven patients were diagnosed by transbronchial biopsy and an open lung biopsy was done in one patient. Patohistological findings revealed lymphoma of marginal zone B cell lymphoma: CD20+/CD10-/CD5-/CyclinD1- /CD23-/IgM- with Ki-67+<20% of all cells. According to the Ferraro staging system, five patients had localized disease (CS I-IIE) and three had stage IVE; bulky tumor mass had 3 patients. All patients had Eastern Cooperative Oncology Group (ECOG) performance status (PS) 0 or 1. Five patients received monochemotherapy with chlorambucil and 3 were treated with CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone). A complete response (CR) was achieved in 5 patients and a partial response (PR) in 3 of them, treated with chlorambucil monotherapy and CHOP regimen. All patients were alive during a median follow-up period of 49 months (range 6- 110 months). Three patients relapsed after monochemotherapy into the other extranodal localization. They were treated with CHOP regimen and remained in stable PR. Conclusion. BALT lymphoma tends to be localised disease at the time of diagnosis, responds well to monochemotherapy with chlorambucil and has a favourable prognosis.Uvod/Cilj. Bronchus-associated lymphoid tissue (BALT) limfom redak je podtip ekstranodusnih B-celijskih limfoma marginalne zone, koji cini 10% svih MALT (mucosa associated lymphoid tissue) limfoma. Ova vrsta limfoma spada u grupu indolentnih limfoma sa lokalizovanom bolescu kod 65-70% bolesnika. Cilj rada bio je retrospektivna analiza toka i ishoda lecenja bolesnika sa ovom vrstom limfoma. Metode. Analizirano je osam bolesnika sa BALT limfomom, dijagostikovanih i lecenih u Institutu za hematologiju Klinickog centra Srbije, u periodu od januara 1998. do aprila 2008. godine. Rezultati. Ispitivanu grupu cinila su dva bolesnika muskog i sest bolesnika zenskog pola, srednje starosti 64 godine (raspon 37-67 godina). Sest bolesnika imalo je nespecificnu respiratornu simptomatologiju, dok su Bsimptomi bili prisutni kod svih ispitanika. Analize za virusne markere antiHCV, antiHIV i HbsAg bile su negativne. Troje bolesnika imalo je Sj?grenov sindrom, reumatoidni artritis i tuberkulozu pluca. Kod sedam bolesnika dijagnoza bolesti postavljena je nakon transbronhijalne biopsije tumorske mase pluca, dok je kod jednog uradjena otvorena biopsija pluca. Patohistoloskom analizom potvrdjeno je da se radi o Bcelijskom non-Hodgkinovom limfomu (B-NHL) marginalne zone pluca sa tipicnim imunofenotipom: CD20+/CD10- /CD5-/CyclinD1-/CD23-/IgM- sa niskim proliferativnim indeksom (Ki-67+ kod manje od 20% celija). Prema modifikovanoj klasifikaciji Ferraro, pet bolesnika imalo je lokalizovanu bolest (klinicki stadijum - KS I-IIE), a troje je bilo u IV stadijumu E KS, sa bulky tumorskom masom kod tri bolesnika. Svi bolesnici imali su Eastern Cooperative Oncology Group (ECOG) performans status (PS) 0 ili 1. Pet bolesnika leceno je primenom monoterpije hlorambucilom, dok je kod tri bolesnika primenjen polihemioterapijski protokol CHOP (ciklofosfamid, doksorubicin, vinkristin i prednizon). Kompletna remisija (KR) bolesti postignuta je kod pet bolesnika, od kojih je cetvoro leceno primenom hlorambucila, dok je kod tri postignut parcijalni odgovor (PR). Medijana pracenja bila je 49 meseci (raspon 6-110 meseci). Kod tri bolesnika nakon primene monoterapije hlorambucilom doslo je do relapsa bolesti u druga ekstranodalna mesta (medijana relapsa 24 meseci, raspon 12-36 meseci), kada je primenjen CHOP protokol sa postizanjem dobrog PR. Zakljucak. Limfome BALT karakterise lokalizovana bolest sa postizanjem KR u velikom procentu nakon primene monoterapije hlorambucilom i CHOP protokola sa dobrom prognozom.