Hairy cell leukemia

Question 1

Review peripheral smear online of HCL

Answer 1

OK

Question 2

clinical features of HCL

Answer 2

Pancytopenia + large palpable spleen + NEVER lymph node involvement/LAD

Question 3

Bone marrow biopsy findings in HCL

Answer 3

• fried egg appearance (see photo online)
• abnormal lymphoid infiltration

Question 4

HCL immunophenotype

Answer 4

CD20 bright and surface immunoglobulin, positivity for CD25, CD11c, annexin A1, and CD103, and (in a large majority of cases) failure to express CD5.

Question 5

Variant HCL features to know

Answer 5

• BRAF negative
• distinct immunophenotype

Question 6

1) Initial management of HCL 2) Indications for treatment

Answer 6

**IF asymptomatic OR none of below → Observation
- Indications for starting treatment:
symptomatic splenomegaly, b symptoms, recurrent infections, cytopenias (hgb<12, platelet count <100, ANC<1)

Question 7

Indications for starting treatment for HCL

Answer 7

• symptomatic splenomegaly
• b symptoms
• recurrent infections
• cytopenias (hgb<12, platelet count <100, ANC<1)

Question 8

Firstline systemic therapy for HCL

Answer 8

*rituxan can be added to purine analogue (deeper response + longer remissions but immunosuppressive – need longer term follow-up)
IF medically fit, cladribine 5-7 day course (High response - more cytopenias but you push through with transfusion support)
IF medically unfit, pentostatin every 2 weeks until progression
IF very frail, rituximab monotherapy

Question 9

Management of relapsed/refractory HCL

Answer 9

IF initial remission >2 yrs, retreat with purine analogue + rituximab
IF remission <2 yrs,
clinical trial
alternative purine analog + rituximab
vemurafenib +/- rituximab