Article
Hematology
Kai Zhu, Andrew Jamroz, Steven Huang, Diego Villa, Ciara L. Freeman, David W. Scott, Graham Slack, Laurie H. Sehn, Joseph M. Connors, Cynthia L. Toze, Kerry J. Savage, Alina S. Gerrie
Summary: Hodgkin variant Richter transformation (HvRT) is a rare and challenging complication of chronic lymphocytic leukaemia (CLL) with limited information on prognostic factors and treatment approaches. In a population-based cohort from British Columbia, poor clinical outcomes were observed in the majority of HvRT patients, although those who received curative-intent therapy had similar survival rates to older patients with de novo Hodgkin lymphoma.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Pathology
Siba El Hussein, Kirill A. Lyapichev, Hong Fang, Fatima Zahra Jelloul, Warren Fiskus, Jiansong Chen, Peng Wei, Ellen Schlette, Jie Xu, Shaoying Li, Rashmi Kanagal-Shamanna, Hong Yang, Zhenya Tang, Beenu Thakral, Sanam Loghavi, Nitin Jain, Philip A. Thompson, Alessandra Ferrajoli, William G. Wierda, Elias Jabbour, Keyur P. Patel, Bouthaina S. Dabaja, Kapil N. Bhalla, Joseph D. Khoury
Summary: Integrated clinicopathological and molecular analyses were performed on 142 cases of RT-DLBCL. The study found that RT-DLBCL has distinctive morphological and immunophenotypic features, including immunoblastic morphology and common expression of CD5, MUM1, and LEF1. Cell-of-origin does not seem to have prognostic implications in RT-DLBCL.
Article
Pathology
Mounir Trimech, Audrey Letourneau, Edoardo Missiaglia, Bernard De Prijck, Monika Nagy-Hulliger, Joan Somja, Manuela Vivario, Philippe Gaulard, Frederic Lambert, Bettina Bisig, Laurence de Leval
Summary: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) can transform into an aggressive disease and increase the risk of developing various types of T-cell lymphoma. We reported a small group of patients with composite lymphomas consisting of CLL/SLL and angioimmunoblastic T-cell lymphoma (AITL), a previously unrecognized association.
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2021)
Review
Pathology
Birgitta Sander, Elias Campo, Eric D. Hsi
Summary: This manuscript reviews the clinicopathologic and biologic features of chronic lymphocytic leukaemia/small lymphocytic lymphoma, B-cell prolymphocytic leukaemia, and mantle cell lymphoma. Discussions focus on incorporating new knowledge into the next classification system.
Article
Pathology
Ambrus Gango, Richard Kiss, Peter Farkas, Eid Hanna, Judit Demeter, Beata Deak, Dora Levai, Lili Kotmayer, Donat Alpar, Andras Matolcsy, Csaba Bodor, Zoltan Matrai, Botond Timar
Summary: Richter syndrome (RS) is a challenging complication with poor prognosis in CLL or SLL patients, characterized by development of high-grade lymphoma. Rare phenotypes of RS can occur during treatment with targeted therapies, and genetic factors play a key role in the pathogenesis of RS.
Article
Hematology
Alex F. Herrera, Kwang Woo Ahn, Carlos Litovich, Yue Chen, Amer Assal, Qaiser Bashir, Ruthee-Lu Bayer, Melanie Coleman, Zachariah DeFilipp, Nosha Farhadfar, Matthew Greenwood, Theresa Hahn, Mitchell Horwitz, Caron Jacobson, Samantha Jaglowski, Sylvie Lachance, Amelia Langston, Bassam Mattar, Richard T. Maziarz, Joseph McGuirk, Mohammad A. H. Mian, Sunita Nathan, Adrienne Phillips, Kevin Rakszawski, Henrik Sengeloev, Shalini Shenoy, Robert Stuart, Craig S. Sauter, Mohamed A. Kharfan-Dabaja, Mehdi Hamadani
Summary: Hematopoietic cell transplantation (HCT), including autologous and allogeneic HCT, may lead to durable remissions in therapy-sensitive patients with Richter syndrome. Complete response was associated with better outcomes in the allo-HCT cohort, while cytogenetic abnormalities and prior novel therapy did not impact outcomes.
Article
Radiology, Nuclear Medicine & Medical Imaging
Lars Kurch, Andreas Huettmann, Thomas W. Georgi, Jan Rekowski, Osama Sabri, Christine Schmitz, Regine Kluge, Ulrich Duehrsen, Dirk Hasenclever
Summary: In diffuse large B-cell lymphoma, continuous scales such as qPET and Delta SUVmax are more suitable for predicting treatment outcomes based on interim PET scans compared to the ordinal Deauville scale. The positive predictive value curves for qPET and Delta SUVmax were found to be similar, with both methods identifying less than 15% of patients as poor responders. The study suggests that continuous scales offer more accurate outcome predictions in this context.
JOURNAL OF NUCLEAR MEDICINE
(2021)
Review
Oncology
Maria Huguet, Jose-Tomas Navarro, Jose Molto, Josep-Maria Ribera, Gustavo Tapia
Summary: Non-Hodgkin lymphoma (NHL) is a common HIV-related neoplasm, with diffuse large B-cell lymphoma (DLBCL) being the most common subtype. DLBCL in people with HIV presents with aggressive characteristics. The introduction of combined antiretroviral therapy (cART) and the use of cART with chemotherapy have significantly improved the prognosis for people with HIV and DLBCL, approaching that of the general population.
Article
Pathology
Siba El Hussein, L. Jeffrey Medeiros, Stephen K. Gruschkus, Peng Wei, Ellen Schlette, Hong Fang, Fatima Zahra Jelloul, Wei Wang, Warren Fiskus, Rashmi Kanagal-Shamanna, Sanam Loghavi, Hong Yang, Shaoying Li, Jie Xu, Zhenya Tang, Beenu Thakral, Nitin Jain, William G. Wierda, Keyur Patel, Kapil N. Bhalla, Joseph D. Khoury
Summary: Immune checkpoint inhibitors (PD-1 inhibitors) have shown clinical activity in Richter transformation-diffuse large B-cell lymphoma variant (RT-DLBCL), providing a novel therapeutic approach. A study group of 64 RT-DLBCL patients was assessed for expression of PD-1, PD-L1, CD30, and microsatellite instability (MSI), as well as evaluated for immune evasion phenotype (IEP) and PD1-positive tumor-infiltrating lymphocytes (TILs). Brisk PD1+ TIL level was more common in IEP+ tumors, and CD30 expression was significantly more common in IEP+ RT-DLBCL. There was no significant difference in age, sex, or time to transformation between the two groups. Notably, patients with brisk PD1+ TILs had better overall survival.
Review
Hematology
Shazia Nakhoda, Aldana Vistarop, Y. Lynn Wang
Summary: Bruton tyrosine kinase inhibitors (BTKi) have had a significant impact on the treatment of chronic lymphocytic leukaemia (CLL) and non-Hodgkin lymphoma. However, resistance to BTKi remains a challenge, and this review article provides an update on the key clinical data, mechanisms of resistance, and ongoing clinical investigations in CLL, mantle cell lymphoma, and diffuse large B-cell lymphoma (DLBCL).
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Oncology
Warren Fiskus, Christopher P. Mill, Dimuthu Perera, Christine Birdwell, Qing Deng, Haopeng Yang, Bernardo H. Lara, Nitin Jain, Jan Burger, Alessandra Ferrajoli, John A. Davis, Dyana T. Saenz, Wendy Jin, Cristian Coarfa, Craig M. Crews, Michael R. Green, Joseph D. Khoury, Kapil N. Bhalla
Summary: This study reports the characteristics of three newly established patient-derived xenograft models of RT-DLBCL, including genetic alterations, gene expressions, and drug sensitivity. The findings suggest potential novel therapies for RT-DLBCL, including BET protein inhibitors.
Review
Oncology
Idanna Innocenti, Giulia Benintende, Annamaria Tomasso, Alberto Fresa, Francesco Autore, Luigi Maria Larocca, Luca Laurenti
Summary: Chronic lymphocytic leukemia can transform into an aggressive lymphoma called Richter syndrome. Immunogenotypic features of Richter syndrome include unmutated IgHV status, deletion of chromosome 17p or 11q, activation of oncogenes, and inactivation of onco-suppressors. The prognosis for patients with Richter syndrome is very poor, and current treatment options are limited. Understanding the biology of this condition is crucial for personalized treatment and improving survival.
HEMATOLOGICAL ONCOLOGY
(2023)
Article
Oncology
Yucai Wang, Sutapa Sinha, Linda E. Wellik, Charla R. Secreto, Karen L. Rech, Timothy G. Call, Sameer A. Parikh, Saad S. Kenderian, Eli Muchtar, Suzanne R. Hayman, Amber B. Koehler, Daniel L. Van Dyke, Jose F. Leis, Susan L. Slager, Haidong Dong, Neil E. Kay, Rong He, Wei Ding
Summary: Compared to CLL patients, RS patients showed higher expression of PD-L1 in histiocytes and dendritic cells, as well as PD1 in neoplastic B cells in nodal tissue, along with increased infiltration of FOXP3-positive T cells and CD163-positive macrophages. Additionally, peripheral blood T-cell receptor clonality was significantly lower in RS patients, indicating a more diverse T-cell population in RS compared to CLL. These findings suggest that CLL and RS have distinct immune signatures that may impact immunotherapy efficacy.
BLOOD CANCER JOURNAL
(2021)
Article
Cell Biology
Eva A. M. Hesius, Lidia van Laar, Margriet Oosterveld, Annemiek B. van Spriel, Blanca Scheijen, Jan Willem Leeuwis, Henri A. M. Marres, Patricia J. T. A. Groenen, Wendy B. C. Stevens, Ellen van Der Spek, Adriaan J. C. van den Brule, Brigiet M. Hoevenaars, Konnie M. Hebeda, Michiel van den Brand
Summary: LBCL-IRF4 is a newly identified subtype of large B cell lymphoma in the 2017 revised WHO classification, initially reported in children. This study aimed to assess the frequency of IRF4 rearrangements in adult DLBCLs that need to be reclassified as LBCL-IRF4 using FISH. Among 238 DLBCL patients, six (including the index patient) were found to have IRF4 rearrangements, and their immunohistochemical profile was consistent with LBCL-IRF4. This study highlights the importance of considering LBCL-IRF4 in older patients and at different anatomical sites.
Review
Oncology
Allison Barraclough, Eliza Hawkes, Laurie H. Sehn, Sonali M. Smith
Summary: Large B-cell lymphoma is the most common lymphoma and has the highest burden of lymphoma-related deaths. Treatment has aimed to achieve a cure with CHOP and rituximab plus CHOP, but not all patients are cured due to the heterogeneity of the disease.
HEMATOLOGICAL ONCOLOGY
(2023)
Letter
Oncology
Riccardo Moia, Riccardo Dondolin, Maria Stefania De Propris, Donatella Talotta, Samir Mouhssine, Francesca Perutelli, Gianluigi Reda, Veronica Mattiello, Gian Matteo Rigolin, Marina Motta, Jacopo Olivieri, Renato Fanin, Omar Perbellini, Isacco Ferrarini, Francesca Romana Mauro, Ilaria Del Giudice, Luca Laurenti, Annamaria Tomasso, Massimo Gentile, Anna Maria Frustaci, Alessandra Tedeschi, Alessandro Gozzetti, Caterina Stelitano, Carlo Visco, Carol Moreno, Francesco Forconi, Roberto Marasca, Marta Coscia, Davide Rossi, Robin Foa, Gianluca Gaidano
HEMATOLOGICAL ONCOLOGY
(2023)
Review
Oncology
Abdurraouf Mokhtar Mahmoud, Gianluca Gaidano, Samir Mouhssine
Summary: Richter Syndrome is the development of aggressive lymphoma in patients with chronic lymphocytic leukemia. It occurs in 1-10% of CLL patients and is refractory to conventional therapies. Recent research has shown that this syndrome is partly related to immune system dysfunction, suggesting that immunotherapy may be an effective treatment.
Letter
Hematology
Fortunato Morabito, Giovanni Tripepi, Francesca Romana Mauro, Luca Laurenti, Gianluigi Reda, Riccardo Moia, Adalgisa Condoluci, Iolanda Vincelli, Annalisa Chiarenza, Ernesto Vigna, Enrica Antonia Martino, Antonella Bruzzese, Sabrina Mezzatesta, Roberta Laureana, Giovanna Cutrona, Francesco Di Raimondo, Gilberto Fronza, Antonella Zucchetto, Riccardo Bomben, Francesca Maria Rossi, Jacopo Olivieri, Francesco Zaja, Davide Rossi, Gianluca Gaidano, Maria Ilaria Del Principe, Fiorella Ilariucci, Giovanni Del Poeta, Manlio Ferrarini, Antonino Neri, Valter Gattei, Massimo Gentile
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Review
Oncology
Emanuele Zucca, Davide Rossi, Francesco Bertoni
Summary: The three main types of marginal zone lymphoma (MZL) are extranodal MZL of mucosa-associated lymphoid tissue, splenic MZL, and nodal MZL. They share certain karyotype lesions and alterations in the NFkB pathway. However, they differ in the presence of recurrent translocations, mutations affecting the Notch signaling pathway, the transcription factors KLF2 or PTPRD. This review summarizes recent advances in understanding the epidemiology, genetics, and biology of MZLs, as well as the standard management principles.
HEMATOLOGICAL ONCOLOGY
(2023)
Letter
Oncology
Riccardo Bomben, Francesca Maria Rossi, Filippo Vit, Tamara Bittolo, Antonella Zucchetto, Robel Papotti, Erika Tissino, Federico Pozzo, Massimo Degan, Jerry Polesel, Pietro Bulian, Roberto Marasca, Gianluigi Reda, Luca Laurenti, Jacopo Olivieri, Annalisa Chiarenza, Roberta Laureana, Massimiliano Postorino, Maria Ilaria Del Principe, Antonio Cuneo, Massimo Gentile, Fortunato Morabito, Gilberto Fronza, Agostino Tafuri, Francesco Zaja, Robin Foa, Francesco Di Raimondo, Giovanni Del Poeta, Valter Gattei
Article
Health Care Sciences & Services
Mattia Rossi, Chiara Mele, Ruth Rossetto Giaccherino, Letizia Meomartino, Denise Brero, Giulia Marsan, Gianluca Aimaretti, Ezio Ghigo, Loredana Pagano
Summary: This study investigated the potential risk factors for radioiodine treatment and post-treatment recurrence in patients with intermediate-risk differentiated thyroid cancer. The study found that extra-thyroid micro-extension, pT3 staging, and the presence of large, multiple or clinically evident lymph node metastases were the main indicators for referring patients to radioiodine treatment. Early recurrence was identified as the most relevant factor for planning further surveillance.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Francesco Marchi, Chiara Kessler, Daniela Distefano, Lodovico Terzi di Bergamo, Luca Fumagalli, Manuela Averaimo, Emanuele Crupi, Fabio Bergamini, Giorgia Melli, Georg Stussi, Davide Rossi, Claudio Gobbi, Paolo Ripellino, Emanuele Pravata, Dominique E. Kuhlen, Christoph Roecken, Pietro Scarone, Bernhard Gerber, Adalgisa Condoluci
Summary: This study found that amyloid, mostly of the ATTR subtype, was detected in over three quarters of patients undergoing surgery for lumbar spinal stenosis, and it is associated with age and ligamentum flavum thickness. These findings suggest that histopathological examination of the ligamentum flavum may be helpful for future decision making.
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2023)
Article
Hematology
Elaine Y. L. Chung, Giulio Sartori, Maurilio Ponzoni, Luciano Cascione, Valdemar Priebe, Zijun Y. Xu-Monette, Xiaosheng Fang, Mingzhi Zhang, Carlo Visco, Alexandar Tzankov, Andrea Rinaldi, Jacopo Sgrignani, Emanuele Zucca, Davide Rossi, Andrea Cavalli, Giorgio Inghirami, David W. W. Scott, Ken H. H. Young, Francesco Bertoni
Summary: The role of ETS1 phosphorylation at threonine 38, a marker for ETS1 activation, in DLBCL was studied in cellular models and clinical specimens. It was found that p-ETS1 was detected in activated B cell-like DLBCL (ABC), not in germinal centre B-cell-like DLBCL (GCB), and its inhibition could benefit lymphoma patients.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Hematology
Riccardo Moia, Lodovico di Terzi Bergamo, Donatella Talotta, Riccardo Bomben, Gabriela Forestieri, Valeria Spina, Alessio Bruscaggin, Chiara Cosentino, Mohammad Almasri, Riccardo Dondolin, Tamara Bittolo, Antonella Zucchetto, Stefano Baldoni, Ilaria Del Giudice, Francesca Romana Mauro, Rossana Maffei, Annalisa Chiarenza, Agostino Tafuri, Roberta Laureana, Maria Ilaria Del Principe, Francesco Zaja, Giovanni D'Arena, Jacopo Olivieri, Silvia Rasi, Abdurraouf Mahmoud, Wael Al Essa, Bassel Awikeh, Sreekar Kogila, Matteo Bellia, Samir Mouhssine, Paolo Sportoletti, Roberto Marasca, Lydia Scarfo, Paolo Ghia, Valter Gattei, Robin Foa, Davide Rossi, Gianluca Gaidano
Summary: XPO1 mutations in CLL were found to be associated with increased binding sites for transcription factors regulated by pathways emanating from the B-cell receptor. These mutations also resulted in transcriptomic features associated with BCR and cytokine signalling. In a study of 957 early-stage CLL cases, XPO1 mutations were identified as a novel predictor of shorter time to first treatment, independent of immunoglobulin status and early-stage prognostic models.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Editorial Material
Oncology
Erika Tissino, Riccardo Bomben, Valter Gattei, Antonella Zucchetto
Summary: CD49d, the alpha chain of VLA-4 integrin, has a negative impact in CLL patients treated with BTK inhibitors. Evaluating CD49d expression can improve prognostic stratification.
CLINICAL CANCER RESEARCH
(2023)
Review
Oncology
Ilaria Romano, Adalgisa Condoluci, Davide Rossi
Summary: Richter's transformation is a rare condition where aggressive lymphoma develops from underlying chronic lymphocytic leukemia/small lymphocytic lymphoma. Traditional treatment options have limited success, but novel targeted therapies show promising results. Noncovalent Bruton tyrosine kinase inhibitors, T-cell-engaging bispecific antibodies, chimeric antigen receptor T-cells, and conjugated monoclonal antibodies may improve treatment outcomes.
CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
(2023)
Review
Oncology
Andrealuna Ucciero, Federico Pagnoni, Lorenza Scotti, Alessia Pisterna, Francesco Barone-Adesi, Gianluca Gaidano, Andrea Patriarca, Monia Lunghi
Summary: The combination of venetoclax with hypomethylating agents significantly improved the outcome of AML patients unfit for intensive chemotherapy. However, real-world studies showed lower survival rates compared to clinical trials. Further research is needed to determine the cause of this discrepancy.
Article
Cell Biology
Amreen Salwa, Alessandra Ferraresi, Eleonora Secomandi, Letizia Vallino, Riccardo Moia, Andrea Patriarca, Beatrice Garavaglia, Gianluca Gaidano, Ciro Isidoro
Summary: Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous cancer characterized by dysregulated autophagy and apoptosis. The expression of BCL2 and BECN1 is inversely correlated in DLBCL patients, and cells with high BCL-2 expression are resistant to chemotherapy while cells with high BECLIN-1 expression are sensitive. Modulating BECLIN-1-dependent autophagy can affect the prognosis of DLBCL patients and potentially enhance the efficacy of chemotherapy.
