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Histologic transformation in marginal zone lymphomas

2015; Elsevier BV; Volume: 26; Issue: 11 Linguagem: Inglês

10.1093/annonc/mdv368

1569-8041

Annarita Conconi, Silvia Franceschetti, Kathrin Aprile von Hohenstaufen, Gloria Margiotta‐Casaluci, Anastasios Stathis, Alden A. Moccia, Francesco Bertoni, Antonio Ramponi, Luca Mazzucchelli, Franco Cavalli, Gianluca Gaïdano, Emanuele Zucca,

Viral-associated cancers and disorders

Abstract Background Histologic transformation (HT) is a poorly understood event in patients with marginal zone lymphoma (MZL). The aim of this study was to analyze incidence and risk factors for HT in a large series of MZL patients. Patients and methods The studied cohort included 340 MZL patients diagnosed and treated between 1995 and 2012: 157 extranodal MZLs [mucosa-associated lymphoid tissue (MALT) lymphoma, 46%], 85 splenic MZLs (SMZLs, 25%) and 37 nodal MZLs (NMZLs, 11%). Sixty-one patients (18%) had bone marrow infiltration at presentation, with or without detectable involvement of peripheral blood, but without other involved sites; they were considered clonal B-cell lymphocytosis of marginal zone origin (CBL-MZ). Results With a median follow-up of 4.8 years, the median overall survival and progression-free survival of the whole population were 14.5 and 5 years, respectively. HT was observed in 13 cases [3.8%, 95% confidence interval (95% CI) 2%–6.5%]. Elevated lactate dehydrogenase (LDH) at diagnosis was associated with the risk of HT ( P = 0.019). HT occurred in 5% of SMZLs, 4% of MALT lymphomas, 3% of NMZLs and 3% of CBL-MZ ( P = 0.974). The risk of HT was 5% (95% CI 3–9%) at 5 and 10 years after diagnosis and 10% (95% CI 5%–20%) at 12 years. At the time of HT, most patients had high LDH and B symptoms. At a median follow-up of 12 months after HT, 4 of 13 patients died, all for lymphoma-related causes, with a 2-year post-transformation survival rate of 57% (95% CI 13%–86%). Conclusions In this large retrospective series, the risk of HT across all MZL types appeared lower than the one reported for follicular lymphoma.