HCL facts

Question 1

HCL immunophenotype

Answer 1

CD11c, CD103, CD123, CD25- positive
bright expression of CD19, CD20, CD22, CD200
CD5, CD23, CD10 negative
ANXA1-positive in BMB- 97%

Question 2

Relationship between IGHV mutations and prognosis in HCL

Answer 2

IGVH4-34
Poor prognosis

Question 3

Mutation associated with worse prognosis in HCL

Answer 3

P53

Question 4

HCL-V name in WHO

Answer 4

Splenic lymphoma with prominent nucleolus (SLPN)

Question 5

HCL M:F

Answer 5

4:1

Question 6

incidence HCL

Answer 6

0.5/100K

Question 7

BRAF inhibitor effectiveness based on mutation in HCL

Answer 7

Effective in V600E but not in other mutations
important to diagnose the mutation prior to treatment

Question 8

MAP2K1 mutation in HCL-V

Answer 8

50%
common with IGHV4-34
May be a target for MEK1/2 inhibitors
Poor prognosis

Question 9

Prognostic factors in HCL

Answer 9

WBC > 10K with > 5K HCL
LDH
B2MG
CD38
unmutated IGHV
IGHV4-34

Question 10

Cladribine response rates in HCL

Answer 10

80-90% CR

Question 11

Addition of R in 1st line HCL

Answer 11

100% CR
5 year PFS of 95%
high 96% uMRD

Question 12

Vemurafenib SE

Answer 12

rash, skin cancer, arthralgia, ocular, QT prolongation, heaptic/pancreatic enzyme elevation

Question 13

2nd line Tx for HCL

Answer 13

BR
re- Cladribine R (if 2-5 years from previous Tx)
BRAFi +- R
BRAF+ MEC
Moxetuximab

Question 14

Moxetumomab pasudotox

Answer 14

CD22 conjugated toxin
Tx of RR HCL
Phase II
SE of capillary leak syndrome and HUS

Question 15

Tx of RR HCL

Answer 15

R plus vemurafenib
Dabrafenib plus trametinib
Moxetemzumuab (anti CD22)
Ibrutinib (only 17% CR)
Venetoclax ± RT

Question 16

Tx of HCL-V and splenic diffuse red pulp lymphoma (SDRPL)

Answer 16

No good evidence
Tx as HCL
Splenectomy for SDRPL

Question 17

Vemurafenib in HCL effectiveness

Answer 17

90% OR
35% CR
when added to Rituximab
90% CR

Question 18

Monitoring of pts with HCL on vemurafenib

Answer 18

Dermatology examination every month
ECG
LFT

Question 19

Ibrutinib in HCL

Answer 19

Response less frequent, less profound, and less rapid as compared to Moxe and to vemurafenib

Question 20

Mutations/alterations with poor prognosis in HCL

Answer 20

CD38
Unmutated IGHV
IGHV4-34
p53
MAP2K1

Question 21

Management of HCL with active infection

Answer 21

Control infection before cladribine
If not possible- IFN, low-dose pentostatin, vemurafenib as bridging

Question 22

BMB timing after cladribine in HCL

Answer 22

After 4-6 months