Info
treatment of small lymphocytic lymphoma
(NEJM 2020;383:460)
- unless “active disease”: Rai System III/IV, Binet stage C, disease-related sx, progressive disease, AIHA or ITP refractory to steroids, recurrent infections
First-line
w/o del(17p)/TP53 use acalabrutinib or ibrutinib (BTK ❌inhibtors) or venetoclax + rituximab; with del(17p)/TP53 use acalabrutinib or venetoclax ± obinutuzumab; ibrutinib + venetoclax w 88% w/ CR but ↑ tox (NEJM 2019;380:2095)
Second-line & beyond
in general, choose Rx w/ mechanism different from 1st line Rx. BTK ❌inhibtors (eg, zanubrutinib), venetoclax, chemo including fludarabine, chlorambucil, or bendamustine + rituximab. Consider allo-HSCT in relapse.
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HSCT is the only curative Rx. Rx choice balances patient/disease characteristics and goals of care. Different rates of complete remission, time to progression, and toxicities.
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Rx for complications: PCP, HSV, VZV Ppx; AIHA/ITP → steroids; recurrent infxns → IVIg
NEW Drugs
- Bruton’s tyrosine kinase (BTK) ❌inhibtor
- ibrutinib, Acalabrutinib
- BCL2 ❌inhibtor:
- Venetoclax (ABT-199)
- Anti-CD20 monoclonal antibody:
- obinuTUzumab (GA101)
- Phosphatidylinositol-3-kinase (PI3K) ❌inhibtor:
- Anti-CD52:
- Alemtuzumab