Richter's transformation as leptomeningeal infiltration in a chronic lymphocytic leukemia patient receiving venetoclax. Could blood‐brain barrier be a disease “sanctuary” during venetoclax treatment?

Dimou, Maria; Bitsani, Aikaterini; Roumelioti, Maria; Dimitrakopoulou, Aglaia; Iliakis, Theodore; Pardalis, Vasileios; Grafakos, Ioannis; Kalyva, Sotiria; Μαρκοπουλοσ, Αθανάσιος; Kyrtsonis, Marie‐Christine; Panayiotidis, Panayiotis

doi:10.1002/ccr3.4002

Cited by 3 publications

(1 citation statement)

References 13 publications

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“… 3 Novel agents, such as venetoclax, have been reported to be effective against CLL; however, venetoclax was found not to cross the blood-brain barrier in a patient with RS presenting with CNS involvement. 14 Therefore, ibrutinib may be a good therapeutic option for patients with unfit RS and CNS involvement.…”

Section: Discussionmentioning

confidence: 99%

An experience with ibrutinib monotherapy for Richter’s syndrome isolated in the central nervous system

Nato

Nagaharu

Inoue

et al. 2022

J Clin Exp Hematopathol

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Richter’s syndrome (RS) of the central nervous system (CNS) is known to have an extremely poor prognosis. Ibrutinib has been reported to have some activity in patients with RS, despite its poor prognosis. Although ibrutinib crosses the blood-brain barrier, its efficacy in RS patients with CNS involvement remains unknown. Here, we report a case of RS isolated in the CNS that was confirmed to be clonally related to chronic lymphocytic leukemia (CLL) by immunoglobulin heavy chain gene analysis. Although the median survival of patients with RS clonally related to CLL was significantly shorter than that of patients with RS clonally unrelated to CLL, the patient received ibrutinib monotherapy without experiencing any significant adverse events, and the disease remained stable with ibrutinib until 6 weeks later. Following whole-brain radiation therapy (40 Gy in 20 fractions) with dexamethasone, the patient has survived for five months after diagnosis. Thus, ibrutinib may be a safe and effective therapeutic option for patients with RS and CNS involvement.

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Section: Discussionmentioning

confidence: 99%