Chronic Lymphocytic Leukemia (CLL)

FeedbackChronic Lymphocytic Leukemia (CLL)- Chronic Relapsing RemittingMost  common leukemia in adultsIncurable but treatable*Remember Supportive Care*Median age of 72M > F80% incidentalSLL: lymphocytes in lymph nodes and spleen instead of blood Presentation: 1) Fatigue2) B symptoms3) High WC4) Cytopenias (Marrow infiltrate, AIHA, ITP, Hyposplenism)Rule out: Reactive (viral serology)- Hepatitis, HIVInvestigate: 1) FBC + blood film (mature lymphocytes) w/ trend2) Haemolysis screen + Coombs test3) B2 Microglobulin (prognostic marker)4) IGs + serum electrophoresis5) Flow cytometry (immunophenotyping)6) LN Bx (core) especially if lymphadenopathy (*SLL)7) BMBx8) Tp53 (17p del or mut)9) CTNTAP if treatment indicated Confirm : 1) Lymphocyte morphology (blood film)- monomorphic mature small w/out nucleolous r/o aggressive pro-lymphocytes r/o prominent nucleoli in reactive lymphocytosis2) 5x10^9 / L circulating clonal B cells for over 3 months on Flow…if below, need annual FBC monitoring 3) Immunophenotype scoring out of 5 (1 point each): +ve CD5, +ve CD23, weak IG expression, absent/weak CD22, absence of FMC-7...need 4 or 5/5 Staging : 1) BINIT A/B/C2) RAI 0-4Prognosis: CLL International Prognostic Index1) Age2) B2 Microglobulin3) TP53 status- continuous therapy better if TP53 mut4) IGHV mutation- better if present5) RAI or BINITThe International Working Group for CLL (iwCLL) treatment- Risk vs. Benefit    1) Cytopenias Hb <100, Plts <100    2) Bulky disease >10cm LN length    3) Constitutional sx disease related    4) AIHA (10-20%) and ITP (2-5%)    5) Symptomatic or fnxnal extranodal involvement    6) Massive Splenomegaly >6cm from costal margin or progressive SM + Sx    7) Lymphocyte doubling time < 6 months or  > 50 percent rise in 2 months  WATCH and WAIT if not reaching criteriaInfection risk (bacterial):   1)   Vaccination (NB: NO LIVE VACCINES)   2)   IVIG in immune paresis   3)   Prophylactic Abx (azithromycin)   4)   PCP prophylaxis while on treatmentNeed IRRAD blood productsTrials for Traditional treatments vs. BTK1 and BCL21) Alliance 2) Mayo clinic study3) FLARE4) CLL14 (German) and Illuminate trials Treatment:1) Traditional:  FCR2) Targeted:    a.    1st gen BTK1- Ibrutinib 420mg OD (continuous)    b.    2nd gen BTK1- Acalabrutonib 100mg BD (continuous)    c.    BCL-2 inhibitor- Venetoclax 400mg OD            i.       Frontline : OVen.. Ven + Obinutuzumab (CD20 mAb) - 1 yr            ii.       Relapse: VenR... Ven + Rituximab (CD20 mAb)- 2 yrs    d.   PI3K inhibitor- Idelalisib - 3rd line as bridging for 2x refractory/2x exposed*High Risk*: Doublet therapy (targeted combined)i. Jain et al. – Phase 2 trialii. CAPTIVATE trial: Ibru + Ven <65,  TP53 mut/del OR unmutated IGHV TRIPLET Therapy trials-ALLO SCT- early referrals in high risk with first progression·   Primary Progression: <6m of response·   Relapse: >6m respon '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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