Here’s how readers responded to a You Make the Call question about treatment of early-relapsing hairy cell leukemia variant.
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HCLV is well known to be BRAF-negative and to have worse outcomes than classic HCL, so vemurafenib is not a good option. Giving rituximab as monotherapy wouldn’t be appropriate, nor would adding cladribine when relapse occurred within 6 months. Bendamustine has been reported to have good overall response rates in a few cases, but, given the patient’s age, there are few case reports proving that ibrutinib would be a suitable option with less toxicity (but still requiring vigilance for atrial fibrillation).
Emmanuel Almanza Huante, MD
Mexico City, Mexico
Rituximab with pentostatin could be another option, because pentostatin works differently from cladribine. Moxetumomab pasudotox-tdfk appears too risky at this age.
Radu Gologan, MD, PhD
For patients with HCL relapsing after 3 to 5 years, chemoimmunotherapy with cladribine and rituximab could be an option. However, in this case with early relapse, the combination of bendamustine with rituximab should be considered instead. Ibrutinib and venetoclax seems to represent an alternative in relapsed/refractory HCL including HCLV. BRAF V600E−positive HCL could also receive BRAF inhibitors (vemurafenib, dabrafenib) either as monotherapy or together with MEK inhibitors (trametinib), but this patient does not have BRAF mutation. Anti-CD22 immunotoxins (moxetumomab pasudotox) are a new therapeutic option in patients with HCL without BRAF V600E and HCL variants.
João Tadeu Damian Souto Filho, MD, PhD
Rio de Janeiro, Brazil
I would recommend 4 doses of weekly rituximab, followed by a Bruton tyrosine kinase inhibitor.
Rahul Naik, MD
Santa Clarita, CA