HCL facts Flashcards
HCL immunophenotype
CD11c, CD103, CD123, CD25- positive
bright expression of CD19, CD20, CD22, CD200
CD5, CD23, CD10 negative
ANXA1-positive in BMB- 97%
Relationship between IGHV mutations and prognosis in HCL
IGVH4-34
Poor prognosis
Mutation associated with worse prognosis in HCL
P53
HCL-V name in WHO
Splenic lymphoma with prominent nucleolus (SLPN)
HCL M:F
4:1
incidence HCL
0.5/100K
BRAF inhibitor effectiveness based on mutation in HCL
Effective in V600E but not in other mutations
important to diagnose the mutation prior to treatment
MAP2K1 mutation in HCL-V
50%
common with IGHV4-34
May be a target for MEK1/2 inhibitors
Poor prognosis
Prognostic factors in HCL
WBC > 10K with > 5K HCL
LDH
B2MG
CD38
unmutated IGHV
IGHV4-34
Cladribine response rates in HCL
80-90% CR
Addition of R in 1st line HCL
100% CR
5 year PFS of 95%
high 96% uMRD
Vemurafenib SE
rash, skin cancer, arthralgia, ocular, QT prolongation, heaptic/pancreatic enzyme elevation
2nd line Tx for HCL
BR
re- Cladribine R (if 2-5 years from previous Tx)
BRAFi +- R
BRAF+ MEC
Moxetuximab
Moxetumomab pasudotox
CD22 conjugated toxin
Tx of RR HCL
Phase II
SE of capillary leak syndrome and HUS
Tx of RR HCL
R plus vemurafenib
Dabrafenib plus trametinib
Moxetemzumuab (anti CD22)
Ibrutinib (only 17% CR)
Venetoclax ± RT
Tx of HCL-V and splenic diffuse red pulp lymphoma (SDRPL)
No good evidence
Tx as HCL
Splenectomy for SDRPL
Vemurafenib in HCL effectiveness
90% OR
35% CR
when added to Rituximab
90% CR
Monitoring of pts with HCL on vemurafenib
Dermatology examination every month
ECG
LFT
Ibrutinib in HCL
Response less frequent, less profound, and less rapid as compared to Moxe and to vemurafenib
Mutations/alterations with poor prognosis in HCL
CD38
Unmutated IGHV
IGHV4-34
p53
MAP2K1
Management of HCL with active infection
Control infection before cladribine
If not possible- IFN, low-dose pentostatin, vemurafenib as bridging
BMB timing after cladribine in HCL
After 4-6 months
