Biology and Treatment of Hairy Cell Leukemia

Paillassa, Jérôme; Troussard, Xavier

doi:10.1007/s11864-020-00732-0

Cited by 8 publications

(10 citation statements)

References 65 publications

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“…If PNAs remain the established L1 treatment for symptomatic HCL patients, chemoimmunotherapy combining PNAs and rituximab is now recommended in second‐line, while immunoconjugates targeting CD22, BRAF inhibitors and BCR inhibitors should be considered for relapsed or refractory patients. 17 …”

Section: Discussionmentioning

confidence: 99%

“…For these relapse/refractory patients, additional options are now available including recombinant immunoconjugates targeting CD22, BRAF inhibitors and BCR inhibitors. 17 …”

Section: Introductionmentioning

confidence: 99%

“…For these relapse/refractory patients, additional options are now available including recombinant immunoconjugates targeting CD22, BRAF inhibitors and BCR inhibitors. 17 In this article, we present the long-term follow-up results from a subset of the French retrospective HCL cohort for patients with two or more relapses, focusing on overall survival (OS), RFS for patients with three (L3) and four (L4) treatment lines before the appearance of the recent treatments.…”

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confidence: 99%

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Patients with relapsed/refractory hairy‐cell leukemia

Paillassa

Troussard²

2021

Cancer Reports

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Background Hairy cell leukemia (HCL) is a rare chronic B‐cell neoplasm with good long‐term prognosis. First and second‐line therapies include purine nucleoside analogues (PNAs) and rituximab, but until recently, limited alternative options were available for patients with two or more relapses. Aim The aim of this study is to describe our real‐life experience with HCL patients in third and fourth‐line therapies. Methods and Results Data from 49 HCL patients with two or more relapses, including 16 patients with three or more relapses, were collected from the French retrospective HCL cohort covering the period from 1980 until 2011. They were analyzed to assess hematological response, relapse free survival (RFS) and overall survival (OS) after third (L3) and fourth line (L4). The median age at diagnosis was 53 years. PNAs were the most frequently used treatments. As L3 therapy, 29 patients received PNAs (66%) and 15 (34%) other treatments (rituximab [11%] or interferon [7%] alone or in combination [16%]). The distribution of L4 treatments was similar. The overall hematological response rate (OHRR) after L3 was 97% (complete hematological response 86%) with a 40% five‐year cumulative incidence of relapse (CIR), a median RFS of 104 months, and a median OS of 235 months. After L4, the OHRR was 94% with a two‐year CIR of fourth relapse of 27%. Eleven secondary cancers (5‐year cumulative incidence of 12%) were diagnosed in 10 patients. Patients with ≥2 relapses experience frequent further relapses, with increasingly shorter time to next treatment as the number of treatment lines increases. Furthermore, treatment strategies are associated with substantial toxicities. Conclusion All these points lead to the need for novel treatments.

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Section: Discussionmentioning

confidence: 99%

“…For these relapse/refractory patients, additional options are now available including recombinant immunoconjugates targeting CD22, BRAF inhibitors and BCR inhibitors. 17 …”

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

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Patients with relapsed/refractory hairy‐cell leukemia

Paillassa

Troussard²

2021

Cancer Reports

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“…2 Cytoreductive therapy is recommended for high-risk patients (age >60 years and/or history of thrombosis). 3 Hairy cell leukemia (HCL) is a rare lymphoproliferative disease (LPD) with a heavy male predominance. HCL must be differentiated from other similar diseases, including an HCL variant and the splenic diffuse red pulp lymphoma.…”

Section: Introductionmentioning

confidence: 99%

“…In relapsed/refractory patients, PA plus rituximab can be considered; novel effective targeted therapies include BRAF inhibitors, anti-CD22 immunoconjugate moxetumomab, and Bruton tyrosine kinase inhibitors. 3…”

Section: Introductionmentioning

confidence: 99%

BRAFV600E mutation in the wrong place: a case of concomitant polycythemia vera, hairy cell leukemia, and thyroid adenoma

Loscocco

Rotunno

Mannelli

et al. 2021

Tumori

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Background: Polycythemia vera (PV) is one of the Philadelphia-negative myeloproliferative neoplasms (MPN), characterized by a pan-myelosis with an erythroid-predominant proliferation mainly driven by somatic JAK2V617F gain-of-function mutation. Hairy cell leukemia (HCL) is a rare B-cell lineage lymphoproliferative disease (LPD) with a typic immunophenotypic profile. BRAFV600E, leading to constitutive activation of the RAF/MEK/ERK signalling pathway and increased cell proliferation, is identified as the driver mutation in almost all cases. Although the risk of developing an LPD is significantly increased in patients with MPN compared with the general population, few cases of co-occurring PV and HCL are reported to date. BRAF is one of the most frequently mutated oncogenes in human cancer and some point mutations were identified in multiple neoplasms in addition to HCL, including follicular and papillary thyroid adenoma and carcinoma. Case presentation: Here we report a molecular diagnostic challenge in a woman with a concomitant diagnosis of JAK2V617F PV, BRAFV600E HCL, and HRASQ61K thyroid follicular adenoma. Conclusion: In the age of molecular and precision medicine, this case underlines the importance of integrating molecular results with clinical, radiologic, cytologic, and histopathologic investigations.

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