Feedback- Synchronous CNS and systemic lymphoma at initial presentation (treatment-naïve; TN-SCNSL)- CNS relapse without recurrent systemic lymphoma (relapsed isolated CNS lymphoma; RI-SCNSL)- Relapsed concomitant systemic and CNS disease following treatment for systemic lymphoma (RC-SCNSL) Generally hybrid disease Investigations- MRI Head w gadolinium- PET-CT- Testicular US (blood testes barrier influences treatment)- Opthalmoscopy/fundoscopy +/- Vitreal biopsy +/- subretinal aspirate – could need RT- Lymph node Biopsy NB: Worthwhile to remember patient hx re relapses- ?Stereotactic Brain Biopsy w/ Intraoperative rapid cytology and rv of frozen sections......NB: Steroids pre-biopsy may yield non-diagnostic results (1/3 if 7 days steroids)- Correlate with imaging and timescale- LPo Good for leptomeningeal (15%) which can be missed on MRIo CSF protein levels are prognostico Flow cytometry o Cytospino PCR for IGHV rearrangement: sens.Trial: MARIETTA study, or also known as the IE LSG 42- Single arm prospective trial, 75 patients- +/- Steroid pre-phase –>MATRIX + RICE alternating induction x3–>CR/PR ->Carmustine-Thiotepa AutoSCT- Pre-morbid performance status <=3- 2-year overall survival for all of those patients included in the trial just under 50%- NB: cytaribin omissions if poor performance status- RICE (Ritux isophosphamide, carboplatin and etoposide)…NB, peripheral neuropathy and neurotoxicitiy- TN-SCNSL best 70% 2 year PFS- RI-SCNSL 40% 2 year PFS... can also be given just MATRIX- RC-SCNSL 14% 2 year PFS- NB if frail elderly, change MATRIX to MARTAResponse assessment : - TN-SCNSL and RC-SCNSL o Brain MRI +/- Spine every 2 cycleso PET scan every 2-3 cycleso PET and MRI pre-auto, determine least partial responseo End of treatment PET (6-8 weeks post) and MRI- RI-SCNSL : MRI brain +/- spine every 2 cycles…PET only if suspicion of progression elsewhereRelapse post MARIETTA :-BTKi ?compassionate access vs Trial-ZUMA7 trial: CAR-T (anti CD19) NB : ICANS/CRS….Approved for DLBCL 12 relapse within 12 months and primary refractory disease that hasn’t responded- PALLIATIVE CARE NB Immuno-privileged sites :- Primary Intraocular Lymphoma :o Stage w PET, MRI head, US Testeso MATRIX vs MARTA vs PREMAINE as frailty allows (like 1’ CNS) –> AutoSCTo +/- Occular RTo Frail++ +-> Intravitreal MTX- Primary Testicular Lymphomao If 1 testicle involved 1/3 of patients have the other involved tooo US Testes –> Orchidectomy + histopathology…if lymphoma ->imaging and investigations as aboveo LP with above investigations as 1/3 have CNS involvemento ?skin lesions sometimes in testicular lymphomao RCHOP vs RPolaChP + CNS prophylaxis w MTXo Radiotherapy (30gy) to contralateral testes to reduce contralateral Relapse risk and/or BL orchidectomy- fertility discussiono System 'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK. Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning. Email: [email protected] Insta: BasicstoBrilliance X: @basics_2_brill Send us your feedback!

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“What did the council decide on item 42?” — answered with the speaker and the moment.

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