El-Sharkawi, Dima
Mayo Clinic
Royal Marsden NHS Foundation Trust, Sutton, United Kingdom.
Citations 2
Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant
Zent, Clive S. , Tiacci, Enrico , Kreitman, Robert J. , Tadmor, Tamar , Tallman, Martin S., Wörmann, Bernhard J., Andritsos, Leslie A. , Arons, Evgeny , Barrientos, Jacqueline C. , Broccoli, Alessandro , Call, Timothy G., Dearden, C. J., El‐Sharkawi, Dima , Fagarasanu, Andrei, Gladstone, Douglas E. , Gozzetti, A, Hampel, Paul J. , Hermel, David J. , Johnston, James B. , Juliusson, Gunnar, Kipps, T J, Lauria, Francesco, Lozanski, Gerard , Park, Jae H, Polliack, Aaron, Quest, Graeme, R., K. , Rogers, Kerry A. , Saven, Alan, Seymour, John F. , Tam, Constantine S., Troussard, Xavier, Zenz, Thorsten, Grever, Michael Rhodes, Gozzetti, Alessandro , Barrientos, Jacqueline , Blachly, James , Dietrich, Sascha , El-Sharkawi, Dima , Falini, Brunangelo , Forconi, Francesco , Gerrie, Alina S , Gozzetti, Alessandro , Hampel, Paul J , Iyengar, Sunil , Johnston, James B , Parikh, Sameer A , Robak, Tadeusz , Rogers, Kerry A , Seymour, John F , Zinzani, Pier Luigi , Zent, Clive S , Tallman, Martin S. , Wörmann, Bernhard , Banerji, Versha , Bhat, Seema A. , Blachly, James S. , Call, Timothy G. , Demeter, Judit , Falini, Brunangelo , Forconi, Francesco , Gerrie, Alina S. , Juliusson, Gunnar , Kipps, Thomas J. , Lauria, Francesco, Parikh, Sameer A. , Park, Jae H. , Polliack, Aaron, Ravandi, Farhad , Tam, Constantine S. , Troussard, Xavier , Zenz, Thorsten , Zinzani, Pier Luigi , Grever, Michael R.
Blood | April 14, 2026 | Volume: 148 | Issue: 1 | Pages: 31-42
Abstract Hairy cell leukemia (HCL) and HCL variant (HCLv) are distinct, rare, and chronic splenic B-cell lymphomas/leukemias that partially overlap in clinicopathologic presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase–activating mutation in >95% of the patients, usually has excellent responses to chemotherapy with purine analogues, and is also amenable to BRAF inhibitor–based targeted treatments. In contrast, HCLv lacks BRAFV600E mutation, requires combined therapy with purine analogues in addition to rituximab, and generally shows less durable responses. Here, an international team of hematologists, experts on these rare diseases, was convened by the Hairy Cell Leukemia Foundation to update the previous guidelines (published in 2017) by providing a summary of current methods to diagnose and manage patients with HCL and HCLv as well as a prospective on newer targeted therapies to further improve outcomes.
Treatment-related outcomes and patterns of relapse in secondary CNS involvement by large B-cell lymphoma
Alderuccio, Juan Pablo , Baggio, Diva , Han, Sunwoo , Nizamuddin, Imran , Saha, Aditi, Dong, Ning , Wang, Yucai, Cherng, Hua‐Jay J. , Wagner-Johnston, Nina D., Desai, Amrita, Geethakumari, Praveen Ramakrishnan , Moyo, Tamara K. , Sandoval‐Sus, Jose , EPPERLA, NARENDRANATH, Kamdar, Manali, Tavarozzi, Rita , Danilov, A., Tun, H., Munoz, Javier, Narkhede, Mayur , Maraj, Adrian Matthew, Osborne, Wendy , Calvert, Victoria, El‐Sharkawi, Dima , Hilali, Ammar, Collins, Graham P., Elmusharaf, Nagah, Santarsieri, Anna , Karim, Farheen, Monick, Sarah, Trutzer, Iris Margalit, Can, Jones, Ayers, Amy , Feo, Jacopo Calabrese De, Ghosh, Nilanjan, Treitman, Rachel, Okcu, Izel, Hann, William D., Barrett, Aisling , Deshani, Vismay, Cwynarski, K., Wang, Yucai , Saha, Aditi , Ghione, Paola , Khwaja, Jahanzaib , Saha, Aditi , Wang, Yucai , Cherng, Hua-Jay J , Tolu, Seda , Wagner-Johnston, Nina , Ollila, Thomas A , Grover, Natalie S , Koff, Jean L , Ramakrishnan Geethakumari, Praveen , Sandoval-Sus, Jose , Epperla, Narendranath , Wallace, Danielle S , Kamdar, Manali , Tun, Han W , Narkhede, Mayur S , Rhodes, Joanna , Okosun, Jessica , El-Sharkawi, Dima , Collins, Graham P , Linton, Kim M , Santarsieri, Anna , Baidoun, Firas , Monick, Sarah E, Calabrese De Feo, Jacopo , Sharp, John , Ghosh, Nilanjan , Okcu, Izel , Hann, William , Barrett, Aisling , Deshani, Vismay , Kahl, Brad S , Chavez, Julio C , Olszewski, Adam J , Ghione, Paola , Dong, Ning, Wagner‐Johnston, Nina D. , Grover, Natalie S. , Koff, Jean L. , Epperla, Narendranath , Wallace, Danielle , Kamdar, Manali , Tun, Han W. , Muñoz, Javier, Rhodes, Joanna , Prica, Anca A. , Kühnl, Andrea , Maraj, Adrian , Smith, Jeff , Calvert, Victoria, Linton, Kim , Baidoun, Firas , Sharp, John , Kallam, Avyakta , Abeyakoon, Chathuri , Kahl, Brad S. , Olszewski, Adam J. , Cwynarski, Kate
Blood | January 5, 2026 | Volume: 147 | Issue: 16 | Pages: 1814-1827
Abstract Secondary central nervous system (CNS) large B-cell lymphoma (SCNSL) occurs in the de novo setting, as a CNS-isolated relapse, or synchronous (concomitant CNS and systemic) relapse. SCNSL is a devastating event without therapeutic consensus. Thus, we aimed to evaluate treatment outcomes in an international cohort. Progression-free survival (PFS), overall survival (OS), and cumulative incidence of relapse (CIR, estimated using competing-risk models) were reported. Prognostic factors were identified in a 6-month landmark multivariate analysis. Outcomes after thiotepa autologous stem cell transplant (ASCT) and chimeric antigen receptor (CAR) T-cell therapy (CAR-T) delivered at relapse were compared after propensity score matching (PSM). A total of 1139 patients were included in the analysis (de novo: 537; relapsed SCNSL: 602). Two-year PFS estimates were 40.4%, 43.9%, and 16.2% for de novo SCNSL, CNS-isolated relapse, and synchronous relapse, respectively. Patients with CNS-isolated relapse demonstrated low rates of systemic recurrence (24-month CIR, 6%). Thiotepa-ASCT correlated with longer survival in de novo SCNSL (PFS: hazard ratio [HR], 0.57; P = .005; and OS: HR, 0.62; P = .023) and CNS-isolated relapses (PFS: HR, 0.55; P = .002; and OS: HR, 0.39; P< .0001). ASCT (thiotepa or no thiotepa) also associated with improved survival in synchronous relapses (PFS: HR, 0.57; P = .023; and OS: HR, 0.48; P = .019). Higher survival with thiotepa-ASCT than CAR-T was observed after PSM (PFS: HR, 0.45; P = .005 and OS: HR, 0.41; P = .014). These data support thiotepa-ASCT in eligible patients, particularly de novo disease and CNS-isolated relapses. CNS-isolated relapse was infrequently associated with systemic recurrence, supporting treatment regimens adopted from primary CNS lymphoma.