2.阮嘉 治疗外周T细胞淋巴瘤的新药.pptx

standardsoftreatment?Shouldall

JiaRuan,M.D.,Ph.D.

CenterforLymphomaandMyeloma

WeillCornellMedicalCollege

NewYorkPresbyterianHospital

JiangsuInstituteofHematology

FirstAffiliatedHospitalofSoochowUniversity

4/1/2017

NewAgentsforPeripheralTCellLymphoma

5-10%ofallNHLinWesterncountries

Mostcommon:PTCL-nos,angioimmunoblastic,andALCL

Hgherincidence,15-20%ofalllymphomas,inAsia

PTCL:uncommonandheterogenous

Mostcommon:PTCL-nos,NK/TCL,andATLL

JClinOncol2008;26:4124-4130

Diagnosis

5-yrOS

%IPI0/1IPI4/5

PTCL-NOS

AITL

32%

32%

50%

56%

11%

25%

ALCL,ALK-

ATLL

NKTCL,nasal

NKTCL,extranasal

Enteropathy-type

Hepatosplenic

Subcutaneouspanniculitis-like

49%

14%

42%

9%

20%

7%

64%

74%

28%

57%

17%

29%

0

60%

13%

7%

0

0

14%

0

0

PTCL:outcomevariesbysubtypesandIPI

ALCL,ALK+70%90%33%

JClinOncol2008;26:4124-4130

Nodal

•PTCL,NOS

•AITL

•FollicularTCL*

•NodalPTCLwithTFH*•ALCL,ALK+

•ALCL,ALK-

•BreastImplant-assocALCL*

Extranodal

•NK/T-cell,nasaltype

•Enteropathy-

associatedTCL

•

intestinalTCL*

•HepatosplenicTCL

c

c

pi

hi

o

p

epitheliotr

Monomor

Disseminated

•T-cellPLL

•T-cellLGL

•ChroniclymphoproliferativeNKcells

Cutaneous

•Mycosisfungoides•Sezarysyndrome•PrimarycutaneousCD30+LPD

•AggressiveNK-cellleukemia•ATLL

•SystemicEBV+TCL

•PrimarycutaneousALCL

•Lymphomatoidpapulosis

•Subcutaneous

panniculitis-likeT-celllymphoma

•Primarycutaneousgamma-deltaT-cell•Other

WHO2016CLASSIFICATION:MatureNK/T-cellNeoplasms

Blood.2016;127:2375-2390

AddingtotheCHOPbackbone

.CHOP+alemtuzumab

.CHOP+denileukindiftitox

.CHOP+bortezomib

.CHOP+etoposide

UpfrontSCTconsolidation

Movingnovelagentstofrontline

II.DevelopnovelstrategiesforR/Rdiseases

I.Improveefficacyofinitialtherapies

Novelagentsandcombinations

PTCL:UnmetClinicalNeeds

Study