DLBCL


  • Clinically and biologically heterogenous groups
  • Expanded list of disease included in WHO 2008 Classification for DLBCL serves i
    • delineating a more homogeneous group of DLBCL for clinical trials and 
    • facilitating the study of rare variants, which may require specialized approaches 
    • Most DLBCL that do not have specific clinical and pathological variants have been included under DLBCL, NOS in WHO 2008 Classification
    • Gene Expression Profile has identified two principal molecular subtypes of DLBCL with distinct genetic alteration, molecular signaling, and clinical outcomes 
        • Germinal Center B-Cell-Type (GCB)
        • Activated B-Cell-Type
      • Yet, is not used in routine clinical practice due to difficulty in reproducibility
    • WHO 2008 Classification also emphasizes on LOCATION and other clinical features in the diagnosis of certain entities. eg. 
      • Primary Mediasternal Large B-Cell Lymphoma (PMBL)
      • Primary CNC Large B-Cell Lymphoma (Primary CNS DLBCL)
  • Variants of DLBCL in 2008 WHO Classification Lancet 2013

    • Variants of diffuse large B-cell lymphoma in the 2008 WHO classification

      • Centroblastic

      • Immunoblastic

      • Anaplastic

      • Plasmablastic

      • T-cellrich

      • Anaplastic lymphoma kinase-positive

      • CD5-positive

      • Germinal centre B cell

      • Non-germinal centre B cell

      • PrimaryCNS

      • Primary cutaneous, leg-type

      • Mediastinal

      • Intravascular

      • Primaryeffusion

      • Epstein-Barr virus-positive in elderly people

      • With chronic inflammation

      • Lymphomatoidgranulomatosis

      • In human herpes virus-8-associated Castleman’s disease 


  • Treatment: Lancet 2013
    • CHOP with Radiation (Pre-rituximab era)
    • CHOP - R with Radiation (Post-rituximab era). 
      • Rituximab improved 3 yr overall survival (93 % vs. 84 %)
    • 30 - 40 % relapse after first-line chemotherapy requiring salvage therapy
    • Plasmablastic DLBCL is a histological variant seen in HIV patients
      • Involves head and neck
      • Usually does not express CD 20, hence, does not benefit from Rituximab

71 yo M is seen for lf hip pain. skin thickening is noted. ddx;

  


 



ddx
  • dlvcl with skin involvement

  • double hit large cell lymphoma 

  • primary cutanoeus lymphoma 

rx
  • bendamustine–Rituxan. 
  • r chop
  • R–EPOCH  
  • R–ice chemotherapy 

  • Granix 

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