Article
Medicine, General & Internal
Thomas D. Garvey, Matthew J. Koster, Kenneth J. Warrington
Summary: Giant cell arteritis (GCA) is the most common primary systemic vasculitis in adults over 50 years old, and advances in arterial imaging have improved diagnosis and disease subset recognition. While glucocorticoids have been the main treatment for decades, new therapeutic options have emerged, providing clinicians with more choices for managing GCA patients.
MAYO CLINIC PROCEEDINGS
(2021)
Article
Oncology
Elsa Maitre, Edouard Cornet, Veronique Salaun, Pauline Kerneves, Stephane Cheze, Yohan Repesse, Gandhi Damaj, Xavier Troussard
Summary: This study aimed to describe the immunophenotypic profile of hairy cell leukemia (HCL) and HCL-like disorders and found that the immunological profile alone is not sufficient for diagnosis. Integration of morphologic, phenotypic, and molecular data is necessary. The addition of CD26 to improve the specificity of the immunological scoring system for HCL was also suggested. Unsupervised analysis showed potential for distinguishing HCL from HCL-like disorders.
Article
Oncology
Elsa Maitre, Cecile Tomowiak, Benjamin Lebecque, Fontanet Bijou, Khaled Benabed, Dina Naguib, Pauline Kerneves, Edouard Cornet, Pierre-Julien Viailly, Jeffrey Arsham, Brigitte Sola, Fabrice Jardin, Xavier Troussard
Summary: Deep sequencing is essential for the diagnosis and prognosis of hairy cell leukemia (cHCL) and HCL-like disorders. BRAF(V600E) mutation is common in cHCL, while absent in vHCL and SDRPL. MAP2K1 mutations are associated with poor prognosis in vHCL/SDRPL. Some KLF2 genetic alterations may be induced by AID. Clonal evolution can occur in some cHCL cases.
Article
Oncology
Alexia Vereertbrugghen, Ana Colado, Ernesto Gargiulo, Raimundo Fernando Bezares, Horacio Fernandez Grecco, Gregorio Cordini, Maria del Rosario Custidiano, Jean-Hugues Francois, Guy Berchem, Mercedes Borge, Jerome Paggetti, Etienne Moussay, Romina Gamberale, Mirta Giordano, Pablo Elias Morande
Summary: The current standard treatment for hairy cell leukemia (HCL) involves purine analogs like cladribine. Venetoclax, a Bcl-2 inhibitor, shows potential as a therapy for HCL, but its efficacy may be influenced by the microenvironment.
FRONTIERS IN ONCOLOGY
(2021)
Article
Hematology
Xavier Troussard, Elsa Maitre, Edouard Cornet
Summary: Hairy cell leukemia and related disorders are characterized by the presence of hairy cells, specific genetic markers, and different clinical courses. Treatment is tailored based on symptoms and may involve chemotherapy with purine analogs or chemo-immunotherapy with rituximab. Management of relapsed disease may involve the use of BRAF or MEK inhibitors, immunoconjugates, or BTK inhibitors, with the optimal sequencing of treatments still to be determined. Bcl-2 inhibitors could play a significant role in the future.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Editorial Material
Hematology
Michael R. Grever
Summary: In this study, the authors investigated the efficacy of dabrafenib plus trametinib in treating relapsed/refractory BRAF V600E mutation-positive hairy cell leukemia. They enrolled extensively pre-treated patients who had already received standard agents for hairy cell leukemia. The overall response rate to the combination therapy was 89.1%, with 65.5% of patients achieving complete remission. Patients continued treatment until unacceptable toxicity, disease progression, or death occurred.
Review
Oncology
Elsa Maitre, Jerome Paillassa, Xavier Troussard
Summary: This article discusses different diseases in the category of mature B-cell neoplasms, such as HCL, SMZL, SDRPL, and SBLPN, and mentions the mutations and signaling pathways associated with these diseases, as well as introduces some new targeted therapies.
FRONTIERS IN ONCOLOGY
(2022)
Review
Oncology
Julie Tran, Charles Gaulin, Martin S. Tallman
Summary: Hairy cell leukemia variant (HCL-V) is a rare and aggressive B cell lymphoproliferative disorder with distinct clinical and pathological features from classic hairy cell leukemia (HCL-C). It has a higher likelihood of being refractory to conventional purine analog therapies and leads to a poorer prognosis. While there is no specific genetic mutation diagnostic of HCL-V, genetic profiling has identified potential therapeutic targets and prognostic markers. Combination chemoimmunotherapies have shown the best results in HCL-V, but targeted therapies and CAR-T cell therapy are being explored for future advancements.
CURRENT TREATMENT OPTIONS IN ONCOLOGY
(2022)
Review
Oncology
Ernesto Gargiulo, Mirta Giordano, Carsten U. Niemann, Etienne Moussay, Jerome Paggetti, Pablo Elias Morande
Summary: HCL is a rare, incurable hematological malignancy. The tumor microenvironment plays a crucial role in disease progression and treatment efficacy. Understanding the interactions between leukemia cells and their milieu is essential for developing new targeted therapies.
FRONTIERS IN ONCOLOGY
(2023)
Article
Medicine, General & Internal
Sahil Khanna
Summary: Clostridioides difficile infection is the most common cause of infectious diarrhea in hospitals with increasing incidence in the community. Management includes diagnostics, newer antibiotics, antibody treatments, and microbiome restoration therapies.
MAYO CLINIC PROCEEDINGS
(2021)
Article
Medicine, General & Internal
Elizabeth A. Coon, J. Eric Ahlskog
Summary: Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure along with parkinsonism or cerebellar ataxia. Diagnosing and managing MSA can be challenging, requiring collaboration between tertiary care centers and local care providers. While there is no cure for MSA yet, treatment focuses on alleviating the most severe symptoms experienced by the patient.
MAYO CLINIC PROCEEDINGS
(2021)
Review
Hematology
Robert J. Kreitman, Evgeny Arons
Summary: Hairy cell leukemia is a B-cell malignancy driven by the BRAF V600E mutation. Combination therapy with purine analogs and rituximab can achieve higher rates of minimal residual disease eradication. BRAF inhibitors, Ibrutinib, and moxetumomab pasudotox have shown efficacy in inducing remission but may require chronic use to prevent relapse.
Review
Hematology
Ewa Robak, Dorota Jesionek-Kupnicka, Tadeusz Robak
Summary: Skin lesions are common in hairy cell leukemia patients, mostly related to autoimmune or infectious processes, with leukemia cutis being extremely rare. This paper reviews the epidemiology, pathogenesis, clinical symptoms, diagnosis, and treatment approaches for skin lesions in HCL patients. The review was conducted through MEDLINE database search and included articles from 1980 to 2020.
ANNALS OF HEMATOLOGY
(2021)
Article
Hematology
Alessandro Broccoli, Lisa Argnani, Laura Nanni, Carolina Terragna, Elena Sabattini, Giulia Gabrielli, Vittorio Stefoni, Cinzia Pellegrini, Beatrice Casadei, Alice Morigi, Ginevra Lolli, Matteo Carella, Paolo Elia Coppola, Pier Luigi Zinzani
Summary: The treatment of hairy cell leukemia has evolved over time, with purine analogues like cladribine now considered the preferred option. Most patients respond well to first-line treatment, and some patients may enjoy long-lasting complete remission after one course of treatment.
AMERICAN JOURNAL OF HEMATOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Jan-Paul Bohn, Stefan Salcher, Andreas Pircher, Gerold Untergasser, Dominik Wolf
Summary: Classic hairy cell leukemia is a rare B-cell malignancy characterized by pancytopenia and infectious complications. The key activating mutation BRAF V600E has been identified, but additional oncogenic biologic features are still under investigation for HCL transformation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)