Hairy Cell

Question 1

Immunophemptype

Answer 1

CD 19 CD 20 CD 103 CD 123 CD 25 CD 200

Question 2

mutations

Answer 2

BRAF
neg in variant

MAP2K1 in variant

Question 3

Lugano criteria

Answer 3

For indolent lymphoma

no need for PETCT in hairy cell

Question 4

Tx

Answer 4

Cladribine for 5 days
ONE CYCLE
SC or IV

Rituximab deepens and prolongs MRD (good for young pts)

Pentostatin (purine analog)
Vemurafinib/Debrafinib (BRAF)
Both for fragile pts
Trametinib (anti-MEK)
Moxetumomab (CD22)
Ibrutinib

Question 5

Cladribine side effects

Answer 5

Neut fever 80%
Lymphopenia with HSV and PCP

Question 6

Early relapse

Answer 6

2 years

Question 7

Tx of relapse

Answer 7

Vemurafinib + R
Mexotumumab pseudotox (anti CD22)

Question 8

Vemurafinib

Answer 8

SJS
skin sensitivity to light
Hepatotoxicity

Question 9

Classic type- clinical manifstations

Answer 9

Indolent, BM and spleen, pancytopenia, monocytopenia, vasculitis, AI.
BM fibrosis, remember to do tap smear

Question 10

Classic type immunophenotype

Answer 10

CD11c CD2, CD22, CD25
CD103, CD123, CD200
Annexin A1, Cyclin D1, FMC7, TBX21

Question 11

Variant type- Clinical manifestations

Answer 11

Leukocytosis, anemia, Thrombocytopenia
does not respond to single agent cladribine

Question 12

cHCL/HCL-v immuphenotype

Answer 12

in HCL-v:
CD25 neg
CD123 neg
CD200 neg
Annexin A1
BRAF V600E

P53 MAp2K1 positive

Question 13

Response rate for 1st line cladribine

Answer 13

75-90%
But 30-40% relapse rate

Question 14

Definition of CR

Answer 14

HB > 12
PLT > 100
Neut > 1500
for at least 1 month
+
No HCL in BM
+
Resolution of organomegaly

Question 15

M:F

Answer 15

4:1

Question 16

Mean age

Answer 16

55