Effective upfront treatment with low-dose ibrutinib for a patient with B cell prolymphocytic leukemia
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SHORT REPORT
Effective upfront treatment with low-dose ibrutinib for a patient with B cell prolymphocytic leukemia Satoko Oka 1 & Kazuo Ono 2 & Masaharu Nohgawa 1 Received: 15 December 2019 / Accepted: 17 January 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Summary 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. Keywords Ibrutinib . B cell prolymphocytic leukemia (B-PLL) . Upfront
Introduction B cell prolymphocytic leukemia (B-PLL) is a rare, aggressive, mature lymphoid neoplasm that accounts for approximately 1% of lymphocytic leukemias and is associated with poor survival (median survival of 3 years after diagnosis) [1]. Although it may be preceded by an indolent phase lasting from months to years, disease progression in B-PLL is generally rapid [2]. Median age at the diagnosis of B-PLL is 69 years. Patients generally present with B symptoms, marked lymphocytosis, splenomegaly, and minimal lymphadenopathy. Its diagnosis requires the presence of prolymphocytes, exceeding 55% of lymphoid cells, in the peripheral blood, characterized by medium-sized cells with clumped chromatin a nd pr o m i n e n t c e nt r a l n uc l e o l i , a nd t h e ty pi c al immunophenotype with the strong expression of B cell antigens CD19, CD20, CD22, and CD79a, with CD5 and CD23 * Satoko Oka [email protected] 1
Division of Hematology, Japanese Red Cross Society Wakayama Medical Center, Wakayama, Wakayama, Japan
2
Division of Pathology, Japanese Red Cross Society Wakayama Medical Center, Wakayama, Japan
expression in 10–30% of cases. More than 50% of patients with B-PLL carry abnormalities in the TP53 tumor suppressor gene and/or a complex karyotype, which are associated with adverse outcomes in chronic lymphocytic leukemia (CLL) [1, 3, 4] and B-PLL has a poorer survival outcome than CLL. Strong resistance to purine analogues/alkylator-based therapy has been reported among B-PLL [5, 6]. Although alemutuzumab is the only effective therapeutic option for these patients, it has been associated with high rates of infections and short-lived responses [7]. In CLL, B cell receptor (BCR) inhibitors (ibrutini
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