b'VA Has More Options as Treatment for Mantle Cell Lymphoma Expands RapidlyMCL Is a Presumptive Condition for Veterans Exposed to Agent OrangeBy Annette M. BoyleMost patients with mantle cell lymphoma, which is consideredWhen people think about mantle cell a presumptive condition for veterans exposed to Agent Orange,lymphoma,theyusuallythinkabout the aggressive subtype, according to face a rapidly progressive disease and higher mortality rates.Michael Kolodziej, MD, vice president New therapies are giving VA clinicians more options to helpand chief innovation officer at ADVI HealthinWashington,DC,inadis-MCL patients. For example, the availability now of three BrutoncussionofnewNationalCooperative tyrosine kinase [BTK] inhibitors is allowing treatment to beCancerNetworkguidelinesforthe disease.Andtheaggressivesubtype better matched to the needs of the patient.ofmantlecelllymphomaistreated muchlike,interestingly,aggressive lymphomaslikediffuselargeB-cell lymphoma. 2SYRACUSE, NYThe last few yearsMCL.Aboutthree-quartersofMCLForpatientswhoaretransplanteli-have seen a dramatic shift in treatmentpatients are male and the average agegible, the goal is to have them achieve for mantle cell lymphoma as multipleof diagnosis is 68 years. remission with intensive induction and new drugs gained approval.While some patients develop an indo- consolidationtherapiesandthenpro-Mantlecelllymphoma(MCL)lent form of MCL, most face a rapidlyceed to autologous stem cell transplant accountsforbetween2%and6%ofprogressive disease with shorter dura- (autoSCT)followedbymaintenance allnon-Hodgkinlymphomas(NHLs).tions of response to therapies as welltherapy with rituximab. For those who Until recently, MCL was simply con- as shorter periods of progression-freeare not transplant eligible, the hope is sidered a subtype of chronic lympho- survivalandoverallsurvivalcom- to knock the disease back as long as cyticleukemiawithparticularlypoorpared to other lymphomas. Low-riskpossible. Bendamustine plus rituximab prognosis.Increasedunderstandingpatients have a five-year survival ratehas been the most common therapy for of the diseases molecular biology hasof 60%.these patients as clinical trials demon-enabled development of new drugs thatIn contrast, intermediate risk patientsstrated longer progression-free survival target specific pathways in MCL, offer- have a median overall survival of 51compared to the two other most com-ing better outcomes with lower rates ofmonths, and those with high-risk diseasemon combinations in elderly patients.toxicity. have overall survival of just 29 months,Bothgroupsfaceahighrateof Thats particularly good new for vet- noted Jeffrey J. Pu, MD, PhD, associaterelapse, however, as there is no curative erans. Asanon-Hodgkinlymphoma,professor of medicine, pathology, andtreatment for MCL. MCL is considered a presumptive con- pharmacology, Upstate Cancer Center, ditionforveteransexposedto AgentState University of New York UpstateBTK sc hangeT reaTmenTOrange.Theolderageandpredomi- Medical University, and Syracuse VAUntilrecently,treatmentofrelapsed nantly male demographic of U.S. vet- Hospital,bothinSyracuse,NY,inaandrefractorymantlecelllymphoma erans also put them at greater risk forreview of the disease. 1 wasreallynotparticularlygood.But 2020 COMPENDIUM OF FEDERAL MEDICINE 11'