title: Blastoid variant of mantle cell lymphoma: late progression from classical mantle cell lymphoma and quantitation of minimal residual disease.
abstract: OBJECTIVE
  Classical mantle cell lymphoma (MCL) and its blastoid variant (MCL-BV) are characterized by an extremely poor prognosis. Long-time survivors are rare, only very few patients with an overall survival over 10 years have been reported. We present a case of a 41-year-old male with a 12 yr history of MCL stage I to show, that very late relapses in MCL are possible and may present as a transformation into an aggressive blastoid variant and to illustrate the value of quantitative minimal residual disease (MRD) monitoring for treatment guidance.
  
  
  METHODS
  Diagnostic lymph node and bone marrow samples were investigated by immunohistochemistry. Clonality analysis was performed by immunoglobulin heavy chain gene (IGVH) and t(11;14) PCR. The MRD assessment was done by real-time quantitative PCR (RQ-PCR) on available follow-up samples.
  
  
  RESULTS
  By histologic review and sequencing of the clonal IGVH and t(11;14) PCR products we demonstrated a common clonal origin of the leucemic MCL-BV and the classical MCL diagnosed 12 yr earlier. Quantitative MRD assessment revealed significant MRD levels after intensive conventional chemotherapy including Rituximab. Therefore, treatment was early intensified by myeloablative radio-chemotherapy and allogeneic peripheral stem cell transplantation from an unrelated HLA-identical donor. This did not translate into a sustained remission as reflected by persisting MRD levels after transplantation and the patient died from rapid progressive disease 3.5 months after transplant.
  
  
  CONCLUSIONS
  This report presents a rare case of long-term survivor of MCL with a progression of the original MCL cell clone to MCL-BV and demonstrates the clinical value of quantitative MRD assessment for optimized therapeutic management.
authors.affiliation: Second Medical Department, University of Schleswig-Holstein, Campus Kiel, Germany. c.pott@med2.uni-kiel.de
authors.forename: Christiane
authors.initials: C
authors.lastname: Pott
authors.forename: Carsten
authors.initials: C
authors.lastname: Schrader
authors.forename: Monika
authors.initials: M
authors.lastname: Brüggemann
authors.forename: Matthias
authors.initials: M
authors.lastname: Ritgen
authors.forename: Lana
authors.initials: L
authors.lastname: Harder
authors.forename: Thorsten
authors.initials: T
authors.lastname: Raff
authors.forename: Markus
authors.initials: M
authors.lastname: Tiemann
authors.forename: Peter
authors.initials: P
authors.lastname: Dreger
authors.forename: Michael
authors.initials: M
authors.lastname: Kneba
mesh_terms: D000328:Adult; D001483:Base Sequence; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D004273:DNA, Neoplasm / Q000235:genetics; D017809:Fatal Outcome; D005803:Genes, Immunoglobulin; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000209:etiology / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D008297:Male; D036102:Peripheral Blood Stem Cell Transplantation; D013997:Time Factors; D014178:Translocation, Genetic
chemical_list: D004273:DNA, Neoplasm