Clinico-biological features and outcome of patients with splenic marginal zone lymphoma with histological transformation
BRITISH JOURNAL OF HAEMATOLOGY(2022)
摘要
We describe 36 patients with splenic marginal zone lymphoma (SMZL) with transformation (SMZL-T), including 15 from a series of 84 patients with SMZL diagnosed at the Hospital Clinic of Barcelona (HCB) and 21 diagnosed with SMZL-T in other centres. In the HCB cohort, the cumulative incidence of transformation at 5 years was 15%. Predictors for transformation were cytopenias, hypoalbuminaemia, complex karyotype (CK) and both the Intergruppo Italiano Linfomi (ILL) and simplified Haemoglobin, Platelet count, lactate dehydrogenase (LDH) and extrahilar Lymphadenopathy (HPLL)/ABC scores (P < 0 center dot 05). The only independent predictor for transformation in multivariate analysis was CK [hazard ratio (HR) 4 center dot 025, P = 0 center dot 05]. Patients with SMZL-T had a significantly higher risk of death than the remainder (HR 3 center dot 89, P < 0 center dot 001). Of the 36 patients with SMZL-T, one developed Hodgkin lymphoma and 35 a diffuse large B-cell lymphoma, 71% with a non-germinal centre phenotype. The main features were B symptoms, lymphadenopathy, and high serum LDH. CK was observed in 12/22 (55%) SMZL-T and fluorescence in situ hybridisation detected abnormalities of MYC proto-oncogene, basic helix-loop-helix transcription factor (MYC), B-cell leukaemia/lymphoma 2 (BCL2) and/or BCL6 in six of 14 (43%). In all, 21 patients received immunochemotherapy, six chemotherapy, one radiotherapy and three splenectomy. The complete response (CR) rate was 61% and the median survival from transformation was 4 center dot 92 years. Predictors for a worse survival in multivariate analysis were high-risk International Prognostic Index (HR 5 center dot 294, P = 0 center dot 016) and lack of CR (HR 2 center dot 67, P < 0 center dot 001).
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关键词
splenic marginal zone lymphoma, histological transformation, complex karyotype, prognostic factors, survival
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