Secondary CNS Lymphoma

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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