Primary CNS Lymphoma

FeedbackCNS Lymphomas1% of all NHL3% of all Brain tumoursMost common subtype (90%) is DLBCL Clinical division:1.  1* CNS lymphoma, 2.  2* CNS lymphoma- TN-SCNSL- RI-SCNSL- RC-SCNSL3.  Immune deficiency assoc- HIV; better prog. Presentation:  -    SOL Sx -    Raised ICP: morning headaches w N+V-    Neuropsych, Behavioural, Memory, Language-    Focal motor + Stroke Sx-    Seizures-    Visual Sx and uveitis Investigations:-    FBC + Blood film (exclude 2* CNS lymphoma and BM), GFR, U&Es-    LDH (prog.)-    Virology (Hep+HIV)-    IGs, SPEp (paraprotein)-    Stereotactic Brain Bx w/ IO rapid cytology and rv of frozen sectionsNB: Steroids pre-biopsy  ?non-diagnostic results -    LP:.Leptomeningeal*.CSF protein- prognostic.Flow.Cytospin.PCR for IGHV r.-    CT Head-    MRI H (w gadolinium) +/- spine Staging:-R/O systemic lymphoma-PET/CT-US Testes-Opthalmoscopy/fundoscopy +/- Vitreal biopsy +/- subretinal aspirate-?BMBxPre-treatment:-Baseline neuropsych + cognitive ax-Premorbid performance status: ECOG, Echo, GFR, PMHxDx w/o Bx-MRI-Clinical features-Clonal B cells in CSF/Vitreous fluid    and/or   PCR IGHV rearrangementTreatment:Induction main: - MATRIX- younger <70- MARTA- older >65Consolidation: -  Whole brain RT-  BCNU Thiotepa AutoSCT- gold standard if fit...Within 6-8 weeks of the 1st day of final induction: consider for all patients with non-progressive disease (EOT MRI)Trials: IELSG32 study (Leukemia, 2022)- induction + consolidation choices for < 70Induction: 3 arms, MTX + Cyt main-   MATRIX- MTX +Cyt + Thiotepa + Ritux -> AutoSCT…..best choice (4 cycles)...7yr 70% survivalConsolidation: efficacy equal AutoSCT and WB-RT, favoured AutoSCT for Sx....MATRIX regimen available on NSSG:- Dose ++ to cross BBB- Folinic Acid rescue*- IVF till MTX levels <0.1 umol/L (1st lvl 48hrs after MTX)- EF >45%- GFR >50NB: stop co-trimoxazole, penicillins, aspirin, NSAIDs, PPIs (inhibit MTX clearance)- MTX  build up in 3rd spaces- Stem cell harvest post #2- Treatment related mortality 4-7% mostly in #1- Dose reduce Cytaribin (2/3instead of 4 cycles) if pre-morbid, 25-50% totalMARTA study (Blood, Nov 22): fit for autosct and >65-   2x MTX, cytarabin and rituximab ->AutoSCTPRIMAIN study(2017): not fit for autosct >= 651.     4x MTX, Ritux + PO procarbazine2.     6mo of PO procarbazine as maintenance?WB-RT for residual disease-   Palliative if unfit and older:DexTemozolomideWB-RT?IT Chemo in leptomeningealIELSG43 study…  favoured AutoSCT PFS and OS to de-escalation consol. Follow Up:- Response Ax with contrast enhanced MRI scan: 1-2mo after consol.- Rpt MRI every 3-4mo for 2 years ++-       - CR: MRI NAD, normal eye, clear CSF- Stable: <50% decrease, <25% increase- PR: 50% tumor reduction ?persistent CSF- '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!

There are no comments yet.

Please log in to write the first comment.