RT Book, Section
A1 Dreyling, Martin
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1178751475
T1 Mantle Cell Lymphoma
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK hemonc.mhmedical.com/content.aspx?aid=1178751475
RD 2024/04/20
AB SUMMARYMantle  cell  lymphoma  is  a  distinct  subtype  of  non-Hodgkin  lymphoma  with  a  pathognomonic  chromosomal  translocation  t(11;14),  leading  to  constitutive  cyclin  D1  overexpression.  The  clinical  presentation  usually  is  characterized  by  widespread  disease,  occurring  more  often  in  male  patients  older  than  age  60  years.  Despite  high  initial  response  rates,  early  relapses  occur  frequently  after  conventional  chemotherapy,  resulting  in  a  median  survival  time  of  only  3–5  years.  However,  10%  to  15%  of  patients  present  with  a  more  indolent,  chronic  course.  Dose-intensified  treatment  regimens  containing  cytarabine,  rituximab,  and  autologous  stem  cell  transplantation  can  achieve  long-term  remissions  in  patients  fit  enough  to  tolerate  such  aggressive  therapy.  For  the  majority  of  older  adult  patients,  rituximab  maintenance  therapy  can  result  in  prolonged  survival.  Targeted  approaches,  including  proteasome  inhibitors,  immunomodulatory  drugs,  and  inhibitors  of  the  B-cell  receptor  pathway,  have  proven  highly  efficacious  in  patients  with  relapsed  disease  and  should  be  implemented  in  a  multimodal  treatment  plan.