Det er ikke indisert å behandle asymptomatiske pasienter i Binet stadium A (evidensgrad A).
Det anbefales å velge type behandling basert på sykdomsrelaterte og pasientrelaterte risikofaktorer (evidensgrad A).
Som førstelinjebehandling anbefales tidsbegrenset BCL2i-basert behandling for alle unntatt de med del(17p)/TP53 mutasjon. BCL2i gis enten i kombinasjon med BTKi (ca. 15 måneder) eller et anti-CD20 antistoff (ca. 12 måneder) (evidensgrad A).
Kontinuerlig BTKi behandling er aktuelt hvis BCL2i-basert tidsbegrenset behandling er kontraindisert (evidensgrad A).
De med del(17p)/TP53 mutasjon anbefales kontinuerlig BTKi som førstelinjebehandling (evidensgrad A). Som et alternativt kan de behandles med tidsbegrenset målrettet behandling (BCL2i + anti-CD20 antistoff eller BCL2i + BTKi) (evidensgrad A).
For de med mutert IGHV gen uten del(17p)/TP53 mutasjon eller del(11q) kan kjemoimmunterapi vurderes kun hvis tidsbegrenset målrettet behandling ikke kan gjennomføres (evidensgrad A).
Hvis førstelinjebehandling var kjemoimmunterapi bør andrelinjebehandling bestå av målrettet behandling: tidsbestemt BCL2i + anti-CD20 antistoff eller kontinuerlig behandling med BTKi (evidensgrad A).
Ved tidlig progresjon; kortere enn 3 år etter målrettet førstelinjebehandling, anbefales annen målrettet behandling som andrelinjebehandling (evidensgrad A).
Ved sen progresjon etter førstelinjebehandling; mer enn 3 år, anbefales å gjenta samme behandling gitt som førstelinjebehandling (evidensgrad C).
Følgende pasienter <70 år med god funksjonsstatus og uten betydelig samsykelighet kan vurderes for allogen stamcelletransplantasjon. Sykdommen skal være under kontroll og pasienten skal ha respondert med minst partiell remisjon på behandling.
De med del(17p)/TP53 mutasjon eller kompleks karyotype.
De som progredierer tidlig etter adekvat førstelinjebehandling med signalveishemmere.
Autoimmune cytopenier uten aktiv KLL behandles med prednisolon 50 mg x 2 (eventuelt 1 mg/kg/d) i en uke, 25 mg x 3 i en uke og deretter avtrappende dosering alt etter effekt (evidensgrad C).
Rituksimab alene eller i kombinasjon med syklofosfamid er aktuelle alternativ ved wAIHA, erytroaplasi og andre immunmedierte komplikasjoner som ikke responderer på prednisolon (evidensgrad C).
Ved hyppige residiverende bakterielle infeksjoner med kapselkledte bakterier er substitusjonsbehandling med immunglobulin aktuelt hvis vaksinasjon (pneumokokkvaksine) ikke har ført fram og hypogammaglobulinemi foreligger (evidensgrad B).
Årlig vaksine mot influensa og Covid-19 ifølge FHIs anbefalinger. Vaksinasjon mot pneumokokker er også anbefalt (evidensgrad D).
PCP-profylakse anbefales under og minst tre måneder etter behandling med fludarabinbaserte kombinasjonsregimer. PCP profylakse er også anbefalt for de som behandles med idelalisib. Ingen rutinemessig infeksjonsprofylakse til de som behandles med BTKi eller BCL2i.
R-CHOP er et vanlig brukt regime ved DLCBL (evidensgrad C). Ved transformasjon til DLBCL (Richters transformasjon) anbefales konsolidering med allogen eller autolog stamcelletransplantasjon dersom lymfomet er klonalt relatert til KLL (evidensgrad D).
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