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Article ; Online: Unusual clinical presentations of hairy cell leukemia.

Troussard, Xavier

2024

Language	English
Publishing date	2024-01-04
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	80008-9
ISSN	1421-9662 ; 0001-5792
ISSN (online)	1421-9662
ISSN	0001-5792
DOI	10.1159/000536043
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Untangling hairy cell leukaemia (HCL) variant and other HCL-like disorders: Diagnosis and treatment.

Troussard, Xavier / Maitre, Elsa

Journal of cellular and molecular medicine

2023 Volume 28, Issue 3, Page(s) e18060

Abstract: The variant form of hairy cell leukaemia (HCL-V) is a rare disease very different from hairy cell leukaemia (HCL), which is a very well-defined entity. The 5th WHO edition (Leukemia, 36, 2022 and 1720) classification (WHO-HAEM5) introduced splenic ... ...

Abstract	The variant form of hairy cell leukaemia (HCL-V) is a rare disease very different from hairy cell leukaemia (HCL), which is a very well-defined entity. The 5th WHO edition (Leukemia, 36, 2022 and 1720) classification (WHO-HAEM5) introduced splenic lymphomas/leukaemias including four different entities: (1) HCL, (2) splenic marginal zone lymphoma (SMZL) with circulating villous cells in the peripheral blood, (3) splenic lymphoma with prominent nucleolus (SLPN), which replaced HCL-V and CD5 negative B-prolymphocytic leukaemia (B-PLL), and (4) splenic diffuse red pulp lymphoma (SDRPL). All these entities have to be distinguished because of a different clinical course and the need for a different treatment. The diagnosis can be challenging because of complex cases and overlap and/or grey zones between all the entities and needs integrating clinical, histologic, immunophenotypic, cytogenetic and molecular data. We review the diagnostic criteria including clinical, immunophenotypic and molecular characteristics of patients with HCL-V and other HCL-like disorders including HCL, SDRPL, SMZL, B-PLL and the Japanese form of HCL. We also discuss the different criteria allowing us to separate these different entities and we will update the recent therapeutic options that have emerged, in particular the advances with chemoimmunotherapy and/or targeted therapies.
MeSH term(s)	Humans ; Leukemia, Hairy Cell/diagnosis ; Leukemia, Hairy Cell/pathology ; Lymphoma ; Leukemia, Lymphocytic, Chronic, B-Cell ; Spleen/pathology
Language	English
Publishing date	2023-12-14
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2074559-X
ISSN	1582-4934 ; 1582-4934 ; 1582-1838
ISSN (online)	1582-4934
ISSN	1582-4934 ; 1582-1838
DOI	10.1111/jcmm.18060
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Zs.A 5752: Show issues

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Article ; Online: Hairy cell leukemia 2024: Update on diagnosis, risk-stratification, and treatment-Annual updates in hematological malignancies.

Troussard, Xavier / Maître, Elsa / Paillassa, Jérôme

American journal of hematology

2024 Volume 99, Issue 4, Page(s) 679–696

Abstract: Disease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous group of mature lymphoid B-cell disorders characterized by the identification of ... ...

Abstract	Disease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous group of mature lymphoid B-cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment. Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11c, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral bone marrow infiltration and the presence of BRAF Risk stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status. VH4-34 positive HCL cases are associated with a poor prognosis, as well as HCL with TP53 mutations and HCL-V. Treatment: Patients should be treated only if HCL is symptomatic. Chemotherapy with risk-adapted therapy purine analogs (PNAs) are indicated in first-line HCL patients. The use of chemo-immunotherapy combining cladribine (CDA) and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus R, MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22, Bruton tyrosine kinase inhibitors (BTKi), and Bcl-2 inhibitors (Bcl-2i). However, the optimal sequence of the different treatments remains to be determined.
MeSH term(s)	Humans ; Leukemia, Hairy Cell/diagnosis ; Leukemia, Hairy Cell/genetics ; Leukemia, Hairy Cell/therapy ; Proto-Oncogene Proteins B-raf ; Hematologic Neoplasms/diagnosis ; Hematologic Neoplasms/genetics ; Hematologic Neoplasms/therapy ; Immunotherapy ; B-Lymphocytes
Chemical Substances	Proto-Oncogene Proteins B-raf (EC 2.7.11.1)
Language	English
Publishing date	2024-03-05
Publishing country	United States
Document type	Journal Article
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.27240
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Zs.A 1251: Show issues

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Article ; Online: Prise en charge de la dégénérescence maculaire liée à l’âge

Troussard, Xavier

La Revue du praticien

2018 Volume 67, Issue 1, Page(s) 96–100

Abstract: Management of age-related macular degeneration. Age-related macular degeneration (ARMD) is the commonest overall cause of irreversible blindness in patients aged 50 or over in the western world. The disease has been traditionally classified into early ... ...

Title translation	Management of age-related macular degeneration.
Abstract	Management of age-related macular degeneration. Age-related macular degeneration (ARMD) is the commonest overall cause of irreversible blindness in patients aged 50 or over in the western world. The disease has been traditionally classified into early and late stages with its dry and wet forms. Patients with age-related maculopathy should consider taking a dietary supplement such as that used in AREDS. There is no effective treatment for geographic atrophy. Anti-vascular endothelial growth factor (VEGF) therapy has revolutionized the treatment of exudative ARMD. We must not forget visual rehabilitation approaches, such as low-vision rehabilitation and optical aids, while waiting to be able to obtain a restoration of the visual function (opto-genetic therapy, Stem cells, artificial retina...).
MeSH term(s)	Aged ; Angiogenesis Inhibitors/therapeutic use ; Humans ; Middle Aged ; Treatment Outcome ; Vascular Endothelial Growth Factor A/antagonists & inhibitors ; Wet Macular Degeneration/drug therapy
Chemical Substances	Angiogenesis Inhibitors ; Vascular Endothelial Growth Factor A
Language	French
Publishing date	2018-12-03
Publishing country	France
Document type	Journal Article
ZDB-ID	205365-2
ISSN	2101-017X ; 0035-2640
ISSN (online)	2101-017X
ISSN	0035-2640
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Article ; Online: Hairy cell leukaemia with unusual BRAF mutations.

Maitre, Elsa / Macro, Margaret / Troussard, Xavier

Journal of cellular and molecular medicine

2023 Volume 27, Issue 17, Page(s) 2626–2630

Abstract: Hairy cell leukaemia (HCL) diagnosis is based on the morphologic detection of circulating abnormal hairy cells in the peripheral blood and/or bone marrow, an HCL immunological score of 3 or 4 based on the expression of the CD11c, CD25, CD103 and CD123 ... ...

Abstract	Hairy cell leukaemia (HCL) diagnosis is based on the morphologic detection of circulating abnormal hairy cells in the peripheral blood and/or bone marrow, an HCL immunological score of 3 or 4 based on the expression of the CD11c, CD25, CD103 and CD123 and also the presence of a BRAF V600E activating mutation in the B-raf proto-oncogene (BRAF gene) (7q34). When using new generation sequencing of 21 targeted genes in 124 HCL patients, we identified a cohort of 6/124 (2%) patients with unusual BRAF mutations: two patients presented non-V600 mutations (BRAF F595L, BRAF W604L respectively) and four other patients silent BRAF mutations. When using droplet digital PCR (ddPCR) three of the four patients with concomitant BRAF V600E and silent mutation were negative. The respective role of these mutations in the occurrence of HCL or its progression remains to be clarified, but BRAF sequencing is necessary in case of negative BRAF V600E by ddPCR.
MeSH term(s)	Humans ; Leukemia, Hairy Cell/diagnosis ; Leukemia, Hairy Cell/genetics ; Proto-Oncogene Proteins B-raf/genetics ; Mutation/genetics ; Bone Marrow ; Exons
Chemical Substances	Proto-Oncogene Proteins B-raf (EC 2.7.11.1) ; BRAF protein, human (EC 2.7.11.1)
Language	English
Publishing date	2023-08-02
Publishing country	England
Document type	Journal Article
ZDB-ID	2074559-X
ISSN	1582-4934 ; 1582-4934 ; 1582-1838
ISSN (online)	1582-4934
ISSN	1582-4934 ; 1582-1838
DOI	10.1111/jcmm.17890
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Zs.A 5752: Show issues

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Article ; Online: Patients with relapsed/refractory hairy-cell leukemia.

Paillassa, Jérôme / Troussard, Xavier

Cancer reports (Hoboken, N.J.)

2021 Volume 5, Issue 3, Page(s) e1495

Abstract: 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 ... ...

Abstract	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.
MeSH term(s)	Humans ; Leukemia, Hairy Cell/drug therapy ; Leukemia, Hairy Cell/epidemiology ; Peptide Nucleic Acids ; Recurrence ; Retrospective Studies ; Rituximab
Chemical Substances	Peptide Nucleic Acids ; Rituximab (4F4X42SYQ6)
Language	English
Publishing date	2021-07-12
Publishing country	United States
Document type	Journal Article
ISSN	2573-8348
ISSN (online)	2573-8348
DOI	10.1002/cnr2.1495
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review.

Claves, Fabien / Carras, Sylvain / Burroni, Barbara / Maitre, Elsa / Boutonnat, Jean / Troussard, Xavier / Molina, Lysiane

EJHaem

2024 Volume 5, Issue 1, Page(s) 242–246

Abstract: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual ... ...

Abstract	Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual meningeal localization in a 33 years old man who presented with headache, hand paresthesia and visual symptoms. Brain magnetic resonance imaging revealed an occipital meningeal lesion. Diagnostic explorations led to the diagnosis of classical HCL with meningeal localization. After treatment by cladribine and rituximab the patient rapidly improved and is still in complete remission 12 months after end of treatment. The literature review identified 9 other cases of HCL with central nervous system localization (CNS) presenting with brain parenchyma and/or meninges localization. Four out of 9 patients presented with hyperleukocytosis. Most patients experienced good responses with various treatments. Cladribine alone or with rituximab led to complete responses similar to our patient. In our patient, molecular biology revealed KLF2 mutations, which implication in the atypical localization could be suspected but would need dedicated studies. In conclusion, CNS localizations of HCL are rare but can be observed and treatment with cladribine alone or with rituximab appears as an effective strategy.
Language	English
Publishing date	2024-01-09
Publishing country	United States
Document type	Case Reports
ISSN	2688-6146
ISSN (online)	2688-6146
DOI	10.1002/jha2.841
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Article ; Online: The revised guidelines for the diagnosis and management of hairy cell leukaemia and the hairy cell leukaemia variant.

Troussard, Xavier / Grever, Michael R

British journal of haematology

2020 Volume 193, Issue 1, Page(s) 11–14

MeSH term(s)	Antigens, CD ; Humans ; Leukemia, Hairy Cell/diagnosis ; Leukemia, Hairy Cell/therapy
Chemical Substances	Antigens, CD
Language	English
Publishing date	2020-12-23
Publishing country	England
Document type	Journal Article ; Comment
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.17201
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Uh III Zs.182: Show issues

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Article: Updates in hairy cell leukemia (HCL) and variant-type HCL (HCL-V): rationale for targeted treatments with a focus on ibrutinib.

Paillassa, Jérôme / Safa, Firas / Troussard, Xavier

Therapeutic advances in hematology

2022 Volume 13, Page(s) 20406207221090886

Abstract: Hairy cell leukemia (HCL) and HCL-like disorders such as hairy cell leukemia variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL) are rare indolent B-cell malignancies. Purine analogs (PNAs), alone or in association with rituximab (R), are the ... ...

Abstract	Hairy cell leukemia (HCL) and HCL-like disorders such as hairy cell leukemia variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL) are rare indolent B-cell malignancies. Purine analogs (PNAs), alone or in association with rituximab (R), are the standard of care for HCL in the first-line setting. However, PNAs are toxic and patients may become resistant to these drugs. Therefore, new therapeutic strategies are needed. Several recent Plain language summary: Bruton's tyrosine kinase (BTK) inhibitors (BTKi) in hairy cell leukemia (HCL) and variant-type HCL
Language	English
Publishing date	2022-04-13
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2585183-4
ISSN	2040-6215 ; 2040-6207
ISSN (online)	2040-6215
ISSN	2040-6207
DOI	10.1177/20406207221090886
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Article ; Online: Leucémie lymphoïde chronique : changement de paradigme Quel est le rôle du biologiste dans ce nouveau monde ?

Troussard, Xavier

Annales de biologie clinique

2015 Volume 73 Suppl 1, Page(s) 19–21

Title translation	Chronic lymphocytic leukemia: a paradigm shift What is the role of the biologist in this new world?.
MeSH term(s)	Biology ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell/therapy ; Professional Role
Language	French
Publishing date	2015-11
Publishing country	France
Document type	Journal Article
ZDB-ID	2023584-7
ISSN	1950-6112 ; 0003-3898
ISSN (online)	1950-6112
ISSN	0003-3898
DOI	10.1684/abc.2015.1082
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