302 4. Haematology (the case of the samba drum playing grandmother) Flashcards
What is Binet stage for CLL?
The Binet staging system for CLL has 3 stages
stage A – you have fewer than 3 groups of swollen (enlarged) lymph nodes
Stage B – you have 3 or more groups of enlarged lymph nodes
Stage C – you have a low number of red blood cells or platelets
What is the drug Privigen?
IV immunoglobulin as prophylaxis
What is CLL?
Chronic lymphocytic leukaemia
A type of lymphoproliferative disorder that causes splenomegaly and lymphadenopathy
How is CLL diagnosed?
By looking at morphology and immunophenotyping
Staged using Binet staging system
What is the treatment for CLL?
If p53 wild-type:
-First line treatment with chemo-immunotherapy regime FCR:
-Fludarabine, cyclophosphamide, rituximab (anti-CD20 monoclonal Ab)
If less fit then for Bendamustine/Rituximab or Chlorambucil/Obinutuzumab treatment
If p53 mutated or deleted, or relapsed CLL disease then for targeted therapy:
-Ibrutinib (oral BTK inhibitor = Bruton’s tyrosine kinase)
-Venetoclax (oral BCL2 inhibitor = anti-apoptotic protein)
-Idelalisib (oral PI3K inhibitor = B cell receptor signalling protein)
-If CLL is complicated by AIHA or ITP, then treat with steroids (prednisolone)
(AIHA = autoimmune haemolytic anaemia; ITP = immune thrombocytopenic purpura)
Why is the drug Ibrutinib significant?
It was the first type of targeted CLL cell proliferation therapy
-It improved progression and overall survival in CLL (relapsed & treatment naïve)
-Has dramatically altered the natural history of CLL
-It does not lead to eradication of disease so long-term therapy is required
How does the drug Venetoclax work?
It triggers apoptosis of CLL cells (sometimes causing tumour lysis syndrome)
-Results in eradication of detectable MRD in a proportion of patients
Venetoclax approved in 2016 for patients with CLL:
with 17p deletion or TP53 mutation or relapse post-first line chemo-immunotherapy
