• Mantle cell lymphoma signature: CD19+, CD20+, CD5+, CD23-, t(11;14) with overexpression of the cyclin D1 (bcl-1) gene 
  • Leukemic involvement: often more indolent course than without
  
  

  Posted 02/28/17 11:19:10 AM by Matthew Cummings

• Hyperviscosity syndrome: First described by Jan Waldenström in his original 1944 report of 2 patients with macroglobulinemia. 
  • Elevated serum/plasma viscosity (either from circulating Igs in plasma cell dyscrasias or WBCs in hyperleukocytosis)
    • Bleeding (skin/mucosal)
    • Blurred vision, dizziness, HA --> marked retinal venous engorgement resembling hot dogs on a string (“sausaging”)
    • Volume overload
  
  

  Posted 02/28/17 11:21:20 AM by Matthew Cummings

• Indication for PLEX in HVS: ANY symptoms!
  • Should be done prior to or in combination w/ chemotherapy (often steroids)
  
  

  Posted 02/28/17 11:25:29 AM by Matthew Cummings

• Ibrutinib for Relapsed/Refractory MCL (NEJM 2013)
  • Bruton's tyrosine kinase inhibitor
    • BTK: mediator of the B-cell–receptor signaling pathway, chronically activated in MCL
    • Phase 2 study of ibrutinib in  111 patients with relapsed or refractory MCL.
      • Two study arms:  those who had previously received at least 2 cycles of bortezomib therapy and those who had received <2 cycles of bortezomibor no prior bortezomib.
      • Response rate of 68% (75 patients): complete response rate of 21% and partial response rate of 47%; no effect of prior bortezomib. 
      • Overall survival 58% at 18 months.
  
  

  Posted 02/28/17 11:29:27 AM by Matthew Cummings

• For further reading...

  Evidence-based focused review of management of hyperviscosity syndrome (Blood 2012)
  How I treat mantle cell lymphoma (Blood 2009)
  Targeting BTK with Ibrutinib in Relapsed or Refractory Mantle-Cell Lymphoma (NEJM 2013)
  
  

  Posted 02/28/17 11:31:35 AM by Matthew Cummings

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