Effective upfront treatment with low-dose ibrutinib for a patient with B cell prolymphocytic leukemia.

Abstract:

:B cell prolymphocytic leukemia (B-PLL) is a rare and aggressive disease that is associated with poor survival. Although initially asymptomatic patients do not require therapy, most patients will progress and inevitably require treatment. More than 50% of patients with B-PLL carry abnormalities in the TP53 tumor suppressor gene and/or complex karyotype and show resistance to conventional chemotherapy. The efficacy of ibrutinib, a B cell receptor inhibitor, for B-PLL with the TP53 abnormality as second-line therapy was recently demonstrated. We herein report that low-dose ibrutinib as upfront therapy induced a complete response in a B-PLL patient with the TP53 abnormality, whose condition has since remained stable with no recurrence for 12 months. Effective treatments for B-PLL are lacking and given its rarity, prospective comparative therapies are not yet available. This case suggests that upfront therapy with ibrutinib improves the outcome of B-PLL.

journal_name

Invest New Drugs

authors

Oka S,Ono K,Nohgawa M

doi

10.1007/s10637-020-00902-9

subject

Has Abstract

pub_date

2020-10-01 00:00:00

pages

1598-1600

issue

5

eissn

0167-6997

issn

1573-0646

pii

10.1007/s10637-020-00902-9

journal_volume

38

pub_type

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