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
VH4-34
Poor prognosis
mutation associated with worse prognosis in HCL
P53
HCL-V name in WHO
splenic lymphoma with prominent nucleolus (SLPN)
HVL 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%
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
Moxetumomab pasudotox
CD22 conjugated toxin
Tx of HCL
80% ORR
CR of 40%
35% MRD-
SE of capillary leak syndrome and TMA
3rd line Tx of HCL
R plus vemurafenib
dabrafenib plus trametinib
Moxetemzumuab (anti CD22)
Ibrutinib (only 17% CR)
venetoclax ± RT
