title: Transformation and additional malignancies are leading risk factors for an adverse course of disease in marginal zone lymphoma.
abstract: BACKGROUND
  Marginal zone lymphoma (MZL) is a non-Hodgkin lymphoma that occurs as extra nodal, nodal, or splenic. While MZL is generally considered an indolent disease, a substantial percentage of patients follow an unfavorable course. The objective of this retrospective analysis was to identify predictors for a reduced overall survival (OS), or conversely an increased OS.
  
  
  METHODS
  One hundred and ninety-seven MZL patients were analyzed. Apart from assessing previously published risk factors, concomitant morbidity at diagnosis, transformation into aggressive lymphoma, and occurrence of additional malignancies were evaluated.
  
  
  RESULTS
  Next to the known risk factors, i.e. above 60 years of age and elevated serum lactate dehydrogenase (LDH), we demonstrate that transformation into aggressive lymphoma, as well as additional malignancies, are important independent risk factors for a shortened OS in a multivariate analysis, irrespective of the MZL localization. Impressively, in the group of patients lacking LDH elevation, transformation, and/or additional malignancies, only 1 of 63 patients died during follow-up compared with 37 of 87 patients in the high-risk group (HR = 22.8; 95% confidence interval 3.1-167.0; P = 0.002).
  
  
  CONCLUSIONS
  Our analysis proposes novel risk factors and warrants for a continuous follow-up to detect the occurrence of transformation and additional malignancies early on.
authors.affiliation: Department of Hematology, Oncology and Tumor Immunology.
authors.forename: A H
authors.initials: AH
authors.lastname: Meyer
authors.forename: A
authors.initials: A
authors.lastname: Stroux
authors.forename: K
authors.initials: K
authors.lastname: Lerch
authors.forename: J
authors.initials: J
authors.lastname: Eucker
authors.forename: J
authors.initials: J
authors.lastname: Eitle
authors.forename: K
authors.initials: K
authors.lastname: Hohloch
authors.forename: M
authors.initials: M
authors.lastname: Andrzejak
authors.forename: K
authors.initials: K
authors.lastname: Possinger
authors.forename: B
authors.initials: B
authors.lastname: Dörken
authors.forename: A
authors.initials: A
authors.lastname: Pezzutto
authors.forename: C W
authors.initials: CW
authors.lastname: Scholz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002471:Cell Transformation, Neoplastic / Q000378:metabolism; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D007770:L-Lactate Dehydrogenase / Q000097:blood; D018442:Lymphoma, B-Cell, Marginal Zone / Q000097:blood / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase
keywords: additional malignancies; marginal zone lymphoma; risk factors; transformation