Response of relapsed central nervous system hairy cell leukemia to vemurafenib

McDowell, Michael M.; Zhu, Xiaodong; Agarwal, Nitin; Nikiforova, Marina N.; Lieberman, Frank S.; Drappatz, Jan

doi:10.1080/10428194.2016.1177773

Cited by 6 publications

(8 citation statements)

References 16 publications

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“…[49] Another report described response to vemurafenib in a patient with refractory leptomeningeal involvement by HCL. [50] This experience parallels responses to vemurafenib observed in patients with metastatic melanoma bearing BRAF V600E mutations with brain metastases. [51] While cladribine crosses the blood brain barrier, given the suboptimal responses to cladribine observed in the few reported cases of CNS involvement by HCL, vemurafenib might be an early therapeutic consideration.…”

Section: Early Experience and Anecdotal Reports Of Braf Inhibitor Thesupporting

confidence: 53%

“…Progression of chronic myelomonocytic leukemia (CMML) bearing an activating mutation in NRAS has also been observed in a patient with BRAF mutant melanoma treated with vemurafenib, providing further clinical evidence BRAF inhibitor monotherapy can accelerate RAS-driven malignancies. [58] Early reports and individual case reports further provided initial insight into toxicities of vemurafenib in patients with HCL, including development of benign seborrheic keratoses, [45] generalized acneiform rash, [50] moderate arthralgias, [47] and weight loss. [48] The larger phase 2 trials permitted more uniform assessment of the nature, frequency, and severity of vemurafenib toxicities in patients with HCL more homogenously treated and prospectively followed than in the prior anecdotal reports.…”

Section: Toxicities Of Braf Inhibitors In the Treatment Of Hclmentioning

confidence: 99%

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BRAF in the cross-hairs

Geyer

Abdel‐Wahab

Tallman

2019

Expert Review of Hematology

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Introduction: Hairy cell leukemia (HCL) is a rare, chronic B-cell lymphoproliferative disorder characterized by distinctive morphologic features and an indolent clinical course. The discovery of a recurrent activating mutation in BRAF (BRAF V600E) as a disease-defining genetic event in HCL has substantial diagnostic and therapeutic implications. Areas covered: Herein the authors review the role of BRAF V600E and RAF-MEK-ERK signaling in the pathogenesis of HCL, anecdotal clinical reports of BRAF inhibitor monotherapy in management of relapsed or refractory HCL, larger phase 2 trials investigating efficacy of BRAF inhibitor therapy for HCL, adverse effects commonly associated with BRAF inhibitor therapy, including cutaneous toxicity, and mechanisms of therapeutic resistance. Expert opinion: Ongoing and planned studies will help to optimize the use of BRAF inhibitor therapy for HCL by determining efficacy of BRAF inhibition in combination with other antigen targeted or molecularly targeted therapies, and more broadly, to determine how hematologists can best utilize and sequence emerging diagnostic and therapeutic modalities in the care of patients with newly diagnosed and relapsed or refractory HCL.

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Section: Early Experience and Anecdotal Reports Of Braf Inhibitor Thesupporting

confidence: 53%

Section: Toxicities Of Braf Inhibitors In the Treatment Of Hclmentioning

confidence: 99%

BRAF in the cross-hairs

Geyer

Abdel‐Wahab

Tallman

2019

Expert Review of Hematology

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“…He had immediate progression of his disease and was again treated with rituximab and intrathecal methotrexate, which stabilized the disease. At subsequent progression, he was successfully treated with vemurafenib at 960 mg twice daily, which resulted in the resolution of intracranial lesions ( 10 ).…”

Section: Discussionmentioning

confidence: 99%

Case report: A case of classic hairy cell leukemia with CNS involvement treated with vemurafenib

2023

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Hairy cell leukemia (HCL) is a rare mature B-cell lymphoproliferative disorder and most often presents as classic hairy cell leukemia. This entity is characterized by an indolent course and the presence of the BRAF V600E mutation. We report the case of an 80-year-old man with a history of classical hairy cell leukemia who presented with fatigue, dizziness, shortness of breath, blurring of vision, and headache. His initial diagnosis was 9 years prior, and he received treatments with cladribine, pentostatin, and rituximab. The workup showed an elevated white blood cell count with atypical lymphocytes, anemia, and thrombocytopenia. A peripheral blood smear confirmed HCL relapse, and a magnetic resonance imaging (MRI) of the brain showed diffuse, nonenhancing masses in the supratentorial and infratentorial regions of the brain. He was initiated on treatment with vemurafenib, with improvements in his white blood cell count and a recovery of his platelet count and hemoglobin. A repeat MRI of the brain after 3 months showed complete resolution of the lesions. Vemurafenib was discontinued after 6 months, with bone marrow biopsy showing no evidence of residual hairy cell leukemia. There have only been limited reports of HCL involvement in the central nervous system in the literature. Due to the rarity of the condition, it is not clear which treatments can be effective for intracranial disease control. Our report shows the successful use of vemurafenib, resulting in complete remission of relapsed HCL with CNS involvement.

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“…Clinically significant extranodal involvement by HCL is rare, and may occur at presentation or in patients with long‐standing disease . Affected sites include lytic bone lesions, central nervous system, skin, breast and salivary gland . Autopsy studies have revealed leukaemic infiltration in multiple organs, although typically without contribution to morbidity or mortality …”

Section: Discussionmentioning

confidence: 99%

Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues

et al. 2017

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Although uncommon, HCL should be considered in the differential diagnosis of small B cell neoplasms involving nodal/extranodal sites, given the therapeutic implications. In particular, recent discoveries including detection of the BRAF mutation in nearly all cases of HCL and the availability of an antibody to CD103 for use in paraffin-embedded tissues will facilitate the distinction of HCL from other small B cell lymphomas in the nodal/extranodal setting.

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Response of relapsed central nervous system hairy cell leukemia to vemurafenib

Cited by 6 publications

References 16 publications

BRAF in the cross-hairs

BRAF in the cross-hairs

Case report: A case of classic hairy cell leukemia with CNS involvement treated with vemurafenib

Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues

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