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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
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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
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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
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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
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For further reading...
Posted 02/28/17 11:31:35 AM by Matthew Cummings