Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285168
Gregory R Keele, Ariel M Hay, Nadia K Holness, Arijita Jash, Sarah E Ewald, Callan O'Connor, Matthew Vincent, Monika Dzieciatkowska, Angelo D'Alessandro, Gary A Churchill, James C Zimring
{"title":"Co-transfection of murine NXPE2 and murine glycophorin A confers reactivity with Ter-119.","authors":"Gregory R Keele, Ariel M Hay, Nadia K Holness, Arijita Jash, Sarah E Ewald, Callan O'Connor, Matthew Vincent, Monika Dzieciatkowska, Angelo D'Alessandro, Gary A Churchill, James C Zimring","doi":"10.3324/haematol.2024.285168","DOIUrl":"10.3324/haematol.2024.285168","url":null,"abstract":"","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3755-3759"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532708/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141633289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285359
Clifford M Csizmar, Antoine N Saliba, Patricia T Greipp, Hassan Alkhateeb, Kebede H Begna, James M Foran, Naseema Gangat, William J Hogan, C Christopher Hook, Mark R Litzow, Abhishek A Mangaonkar, Jeanne M Palmer, Animesh Pardanani, Mithun V Shah, Ayalew Tefferi, Mehrdad Hefazi Torghabeh, Alexandra P Wolanskyj-Spinner, Mrinal M Patnaik, Scott H Kaufmann, Aref Al-Kali
{"title":"FLT3 inhibitors potentially improve response rates in acute myeloid leukemia harboring t(6;9)(<i>DEK::NUP214</i>): the Mayo Clinic experience.","authors":"Clifford M Csizmar, Antoine N Saliba, Patricia T Greipp, Hassan Alkhateeb, Kebede H Begna, James M Foran, Naseema Gangat, William J Hogan, C Christopher Hook, Mark R Litzow, Abhishek A Mangaonkar, Jeanne M Palmer, Animesh Pardanani, Mithun V Shah, Ayalew Tefferi, Mehrdad Hefazi Torghabeh, Alexandra P Wolanskyj-Spinner, Mrinal M Patnaik, Scott H Kaufmann, Aref Al-Kali","doi":"10.3324/haematol.2024.285359","DOIUrl":"10.3324/haematol.2024.285359","url":null,"abstract":"","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3785-3789"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141426735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285266
Grazia Gargano, Maria Carmela Vegliante, Flavia Esposito, Susanna A Pappagallo, Elena Sabattini, Claudio Agostinelli, Stefano A Pileri, Valentina Tabanelli, Maurilio Ponzoni, Luisa Lorenzi, Fabio Facchetti, Arianna Di Napoli, Marco Lucioni, Marco Paulli, Lorenzo Leoncini, Stefano Lazzi, Stefano Ascani, Giuseppina Opinto, Gian Maria Zaccaria, Giacomo Volpe, Paolo Mondelli, Antonella Bucci, Laura Selicato, Antonio Negri, Giacomo Loseto, Felice Clemente, Anna Scattone, Alfredo F Zito, Luca Nassi, Nicoletta Del Buono, Attilio Guarini, Sabino Ciavarella
{"title":"A targeted gene signature stratifying mediastinal gray zone lymphoma into classical Hodgkin lymphoma-like or primary mediastinal B-cell lymphoma-like subtypes.","authors":"Grazia Gargano, Maria Carmela Vegliante, Flavia Esposito, Susanna A Pappagallo, Elena Sabattini, Claudio Agostinelli, Stefano A Pileri, Valentina Tabanelli, Maurilio Ponzoni, Luisa Lorenzi, Fabio Facchetti, Arianna Di Napoli, Marco Lucioni, Marco Paulli, Lorenzo Leoncini, Stefano Lazzi, Stefano Ascani, Giuseppina Opinto, Gian Maria Zaccaria, Giacomo Volpe, Paolo Mondelli, Antonella Bucci, Laura Selicato, Antonio Negri, Giacomo Loseto, Felice Clemente, Anna Scattone, Alfredo F Zito, Luca Nassi, Nicoletta Del Buono, Attilio Guarini, Sabino Ciavarella","doi":"10.3324/haematol.2024.285266","DOIUrl":"10.3324/haematol.2024.285266","url":null,"abstract":"","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3771-3775"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141633287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2023.284810
Olaf Penack, Christophe Peczynski, William Boreland, Daniel Wolff, Ivan Moiseev, Hélène Schoemans, Christian Koenecke, Charlotte Graham, Zinaida Peric
Chimeric antigen receptor (CAR) T cells are in standard clinical use to treat relapsed or refractory hematologic malignancies, such as non-Hodgkin lymphoma, multiple myeloma and acute lymphoblastic leukemia. Owing to the rapidly progressing field of CAR T-cell therapy and the lack of generally accepted treatment guidelines, we hypothesized significant differences between European centers in prevention, diagnosis and management of short- and long-term complications. To capture the current CAR T-cell management among European Society for Blood and Marrow Transplantation (EBMT) centers and to determine the medical need and specific areas for future clinical research the EBMT Transplant Complications Working Party performed a survey among 227 EBMT CAR T-cell centers. We received complete servey answers from 106 centers (47%) addressing questions in the areas of product selection, CAR T-cell logistics, management of cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome as well as management in later phases including prolonged cytopenias. We identified common patterns in complication management, but also significant variety in clinical management of the centers in important aspects. Our results demonstrate a high medical need for treatment harmonization and future clinical research in the following areas: treatment of steroid-refractory and very severe cytokine release syndrome/neurotoxicity, treatment of cytopenia, early discharge and outpatient management, as well as immunoglobulin substitution.
{"title":"Management of complications of chimeric antigen receptor T-cell therapy: a report by the European Society of Blood and Marrow Transplantation.","authors":"Olaf Penack, Christophe Peczynski, William Boreland, Daniel Wolff, Ivan Moiseev, Hélène Schoemans, Christian Koenecke, Charlotte Graham, Zinaida Peric","doi":"10.3324/haematol.2023.284810","DOIUrl":"10.3324/haematol.2023.284810","url":null,"abstract":"<p><p>Chimeric antigen receptor (CAR) T cells are in standard clinical use to treat relapsed or refractory hematologic malignancies, such as non-Hodgkin lymphoma, multiple myeloma and acute lymphoblastic leukemia. Owing to the rapidly progressing field of CAR T-cell therapy and the lack of generally accepted treatment guidelines, we hypothesized significant differences between European centers in prevention, diagnosis and management of short- and long-term complications. To capture the current CAR T-cell management among European Society for Blood and Marrow Transplantation (EBMT) centers and to determine the medical need and specific areas for future clinical research the EBMT Transplant Complications Working Party performed a survey among 227 EBMT CAR T-cell centers. We received complete servey answers from 106 centers (47%) addressing questions in the areas of product selection, CAR T-cell logistics, management of cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome as well as management in later phases including prolonged cytopenias. We identified common patterns in complication management, but also significant variety in clinical management of the centers in important aspects. Our results demonstrate a high medical need for treatment harmonization and future clinical research in the following areas: treatment of steroid-refractory and very severe cytokine release syndrome/neurotoxicity, treatment of cytopenia, early discharge and outpatient management, as well as immunoglobulin substitution.</p>","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3557-3565"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532686/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285057
Alex Bataller, Hagop Kantarjian, Alexandre Bazinet, Tapan Kadia, Naval Daver, Courtney D DiNardo, Gautam Borthakur, Sanam Loghavi, Keyur Patel, Guilin Tang, Koji Sasaki, Nicholas J Short, Musa Yilmaz, Ghayas C Issa, Yesid Alvarado, Guillermo Montalban-Bravo, Abhishek Maiti, Hussein A Abbas, Koichi Takahashi, Sherry Pierce, Elias Jabbour, Guillermo Garcia-Manero, Farhad Ravandi
Patients with relapsed acute myeloid leukemia (AML) experience dismal outcomes. We performed a comprehensive analysis of patients with relapsed AML to determine the genetic dynamics and factors predicting survival. We analyzed 875 patients with newly diagnosed AML who received intensive treatment or low-intensity treatment. Of these patients, 197 subsequently relapsed. Data were available for 164 of these patients, with a median time from complete remission/complete remission with incomplete blood count recovery to relapse of 6.5 months. Thirty-five of the 164 patients (21%) experienced relapse after allogeneic hematopoietic stem cell transplantation. At relapse, mutations in genes involved in pathway signaling tended to disappear, whereas clonal hematopoiesis-related mutations or TP53 tended to persist. Patients with normal karyotypes tended to acquire cytogenetic abnormalities at relapse. Patients treated intensively had a higher rate of emergence of TP53 mutations (16%), compared to patients given low-intensity treatment (1%, P=0.009). The overall response rates were 38% and 35% for patients treated with salvage intensive treatment or low-intensity treatment, respectively. Seventeen patients (10%) underwent allogeneic stem cell transplantation after salvage therapy. The median overall survival duration after relapse was 5.3 months, with a 1-year overall survival rate of 17.6%. Complex karyotype (hazard ratio [HR]=2.14, P<0.001), a KMT2A rearrangement (HR=3.52, P=0.011), time in remission <12 months (HR=1.71, P=0.011), and an elevated white blood cell count at relapse (HR=2.38, P=0.005) were independent risk factors for overall survival duration. More effective frontline and maintenance therapies are warranted to prevent relapsed AML.
{"title":"Outcomes and genetic dynamics of acute myeloid leukemia at first relapse.","authors":"Alex Bataller, Hagop Kantarjian, Alexandre Bazinet, Tapan Kadia, Naval Daver, Courtney D DiNardo, Gautam Borthakur, Sanam Loghavi, Keyur Patel, Guilin Tang, Koji Sasaki, Nicholas J Short, Musa Yilmaz, Ghayas C Issa, Yesid Alvarado, Guillermo Montalban-Bravo, Abhishek Maiti, Hussein A Abbas, Koichi Takahashi, Sherry Pierce, Elias Jabbour, Guillermo Garcia-Manero, Farhad Ravandi","doi":"10.3324/haematol.2024.285057","DOIUrl":"10.3324/haematol.2024.285057","url":null,"abstract":"<p><p>Patients with relapsed acute myeloid leukemia (AML) experience dismal outcomes. We performed a comprehensive analysis of patients with relapsed AML to determine the genetic dynamics and factors predicting survival. We analyzed 875 patients with newly diagnosed AML who received intensive treatment or low-intensity treatment. Of these patients, 197 subsequently relapsed. Data were available for 164 of these patients, with a median time from complete remission/complete remission with incomplete blood count recovery to relapse of 6.5 months. Thirty-five of the 164 patients (21%) experienced relapse after allogeneic hematopoietic stem cell transplantation. At relapse, mutations in genes involved in pathway signaling tended to disappear, whereas clonal hematopoiesis-related mutations or TP53 tended to persist. Patients with normal karyotypes tended to acquire cytogenetic abnormalities at relapse. Patients treated intensively had a higher rate of emergence of TP53 mutations (16%), compared to patients given low-intensity treatment (1%, P=0.009). The overall response rates were 38% and 35% for patients treated with salvage intensive treatment or low-intensity treatment, respectively. Seventeen patients (10%) underwent allogeneic stem cell transplantation after salvage therapy. The median overall survival duration after relapse was 5.3 months, with a 1-year overall survival rate of 17.6%. Complex karyotype (hazard ratio [HR]=2.14, P<0.001), a KMT2A rearrangement (HR=3.52, P=0.011), time in remission <12 months (HR=1.71, P=0.011), and an elevated white blood cell count at relapse (HR=2.38, P=0.005) were independent risk factors for overall survival duration. More effective frontline and maintenance therapies are warranted to prevent relapsed AML.</p>","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3543-3556"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532689/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140874597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285000
Talha Badar, Ahmad Nanaa, Ehab Atallah, Rory M Shallis, Emily C Craver, Zhuo Li, Aaron D Goldberg, Antoine N Saliba, Anand Patel, Jan P Bewersdorf, Adam Duvall, Madelyn Burkart, Danielle Bradshaw, Yasmin Abaza, Maximilian Stahl, Neil Palmisiano, Guru Subramanian Guru Murthy, Amer M Zeidan, Vamsi Kota, Mrinal M Patnaik, Mark R Litzow
While there is clear evidence to suggest poorer outcome associated with multi-hit (MH) TP53 mutation (TP53MT) compared to a single-hit (SH) mutation in lower-risk myelodysplastic syndrome (MDS), data are conflicting in both higher-risk MDS and acute myeloid leukemia (AML). We conducted an in-depth analysis utilizing data from ten US academic institutions to study differences in molecular characteristics and outcomes of SH (N=139) versus MH (N=243) TP53MT AML. Complex cytogenetics were more common in MH than in SH TP53MT AML (P<0.001); whereas ASXL1 (P<0.001), RAS (P<0.001), splicing factor (P=0.003), IDH1/2 (P=0.001), FLT3 ITD (P<0.001) and NPM1 (P=0.005) mutations clustered significantly with SH TP53MT AML. Survival after excluding patients who received best supportive care alone was dismal but not significantly different between patients with SH or MH disease (event-free survival: 3.0 vs. 2.20 months, respectively, P=0.22; overall survival: 8.50 vs. 7.53 months, respectively, P=0.13). In multivariable analysis, IDH1 mutation and allogeneic hematopoietic stem cell transplantation as a time-dependent covariate were associated with superior event-free survival (hazard ratio [HR]=0.44, 95% confidence interval [95% CI]: 0.19-1.01, P=0.05 and HR=0.34, 95% CI: 0.18-0.62, P<0.001) and overall survival (HR=0.24, 95% CI: 0.08-0.71, P=0.01 and HR=0.28, 95% CI: 0.16-0.47, P<0.001). Complex cytogenetics (HR=1.56, 95% CI: 1.01-2.40, P=0.04) retained an unfavorable significance for overall survival. Our analysis suggests that MH TP53MT is less relevant in independently predicting outcomes in patients with AML than in those with MDS.
虽然有明确的证据表明,在低风险骨髓增生异常综合征(MDS)中,多基因突变(MH)TP53 与单基因突变(SH)相比预后较差,但在高风险 MDS 和急性髓性白血病(AML)中的数据却相互矛盾。我们利用 10 家美国学术机构的数据进行了深入分析,研究了 SH(n= 139)与 MH(n= 243)TP53MTAML 的分子特征和预后差异。与 SH TP53MT AML 相比,复杂细胞遗传学(CG)在 MH 中更为常见(p
{"title":"Prognostic impact of 'multi-hit' <i>versus</i> 'single-hit' <i>TP53</i> alteration in patients with acute myeloid leukemia: results from the Consortium on Myeloid Malignancies and Neoplastic Diseases.","authors":"Talha Badar, Ahmad Nanaa, Ehab Atallah, Rory M Shallis, Emily C Craver, Zhuo Li, Aaron D Goldberg, Antoine N Saliba, Anand Patel, Jan P Bewersdorf, Adam Duvall, Madelyn Burkart, Danielle Bradshaw, Yasmin Abaza, Maximilian Stahl, Neil Palmisiano, Guru Subramanian Guru Murthy, Amer M Zeidan, Vamsi Kota, Mrinal M Patnaik, Mark R Litzow","doi":"10.3324/haematol.2024.285000","DOIUrl":"10.3324/haematol.2024.285000","url":null,"abstract":"<p><p>While there is clear evidence to suggest poorer outcome associated with multi-hit (MH) TP53 mutation (TP53MT) compared to a single-hit (SH) mutation in lower-risk myelodysplastic syndrome (MDS), data are conflicting in both higher-risk MDS and acute myeloid leukemia (AML). We conducted an in-depth analysis utilizing data from ten US academic institutions to study differences in molecular characteristics and outcomes of SH (N=139) versus MH (N=243) TP53MT AML. Complex cytogenetics were more common in MH than in SH TP53MT AML (P<0.001); whereas ASXL1 (P<0.001), RAS (P<0.001), splicing factor (P=0.003), IDH1/2 (P=0.001), FLT3 ITD (P<0.001) and NPM1 (P=0.005) mutations clustered significantly with SH TP53MT AML. Survival after excluding patients who received best supportive care alone was dismal but not significantly different between patients with SH or MH disease (event-free survival: 3.0 vs. 2.20 months, respectively, P=0.22; overall survival: 8.50 vs. 7.53 months, respectively, P=0.13). In multivariable analysis, IDH1 mutation and allogeneic hematopoietic stem cell transplantation as a time-dependent covariate were associated with superior event-free survival (hazard ratio [HR]=0.44, 95% confidence interval [95% CI]: 0.19-1.01, P=0.05 and HR=0.34, 95% CI: 0.18-0.62, P<0.001) and overall survival (HR=0.24, 95% CI: 0.08-0.71, P=0.01 and HR=0.28, 95% CI: 0.16-0.47, P<0.001). Complex cytogenetics (HR=1.56, 95% CI: 1.01-2.40, P=0.04) retained an unfavorable significance for overall survival. Our analysis suggests that MH TP53MT is less relevant in independently predicting outcomes in patients with AML than in those with MDS.</p>","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3533-3542"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532685/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285630
Abdulaziz Kamili, Paymon Ahmadi, Lisa Leypoldt, Franziska Marquard, Christoph Schaefers, Ricardo Kosch, Frederik Peters, Henrik Kusche, Tanja Zamrik, Christine Hanoun, Maximilian Seib, Evgenii Shumilov, Theo Leitner, Cyrus Khandanpour, Carsten Bokemeyer, Katja Weisel, Susanne Ghandili
{"title":"Multiple myeloma in the young: insights on prognosis, clinical features and treatment outcome derived from nationwide German registry data and a nested multicenter sample.","authors":"Abdulaziz Kamili, Paymon Ahmadi, Lisa Leypoldt, Franziska Marquard, Christoph Schaefers, Ricardo Kosch, Frederik Peters, Henrik Kusche, Tanja Zamrik, Christine Hanoun, Maximilian Seib, Evgenii Shumilov, Theo Leitner, Cyrus Khandanpour, Carsten Bokemeyer, Katja Weisel, Susanne Ghandili","doi":"10.3324/haematol.2024.285630","DOIUrl":"10.3324/haematol.2024.285630","url":null,"abstract":"","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3795-3799"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141633292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2024.285903
Ross T Salvaris, Benjamin M Allanson, Graham Collins, Chan Cheah
Recent updates have detailed how patients with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) may be better risk stratified using prognostic scoring systems. Most patients with NLPHL present with early-stage disease and have an indolent disease course. To reflect these differences from classic Hodgkin lymphoma, nomenclature has been updated to recognize nodular lymphocyte-predominant B-cell lymphoma as an alternative to NLPHL. The Global NLPHL One Working Group have published their pivotal dataset in 2024 which challenges the prognostic significance of variant immunoarchitectural (IAP) patterns and proposes a new prognostic scoring system. Key identified prognostic factors include age >45 years, stage III-IV disease, hemoglobin <10.5 g/dL and splenic involvement. After multivariate analysis, variant IAP was not shown to be associated with inferior outcome. As most patients with NLPHL have excellent long-term survival, identifying patients where treatment de-escalation is appropriate will help to minimize toxicity. De-escalation strategies include observation after fully resected stage I disease, active surveillance, anti-CD20 antibody monotherapy, radiotherapy in early-stage disease, and avoiding anthracycline- or bleomycin-containing chemotherapy regimens. Evidence supporting the use of novel therapies remains limited with disappointing results from a recently published study of ibrutinib in patients with relapsed NLPHL. Hopefully, future trials will investigate novel agents such as checkpoint inhibitors, T-cell engaging antibodies and chimeric antigen receptor T-cell therapy.
{"title":"Nodular lymphocyte-predominant Hodgkin lymphoma: advances in disease biology, risk stratification, and treatment.","authors":"Ross T Salvaris, Benjamin M Allanson, Graham Collins, Chan Cheah","doi":"10.3324/haematol.2024.285903","DOIUrl":"10.3324/haematol.2024.285903","url":null,"abstract":"<p><p>Recent updates have detailed how patients with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) may be better risk stratified using prognostic scoring systems. Most patients with NLPHL present with early-stage disease and have an indolent disease course. To reflect these differences from classic Hodgkin lymphoma, nomenclature has been updated to recognize nodular lymphocyte-predominant B-cell lymphoma as an alternative to NLPHL. The Global NLPHL One Working Group have published their pivotal dataset in 2024 which challenges the prognostic significance of variant immunoarchitectural (IAP) patterns and proposes a new prognostic scoring system. Key identified prognostic factors include age >45 years, stage III-IV disease, hemoglobin <10.5 g/dL and splenic involvement. After multivariate analysis, variant IAP was not shown to be associated with inferior outcome. As most patients with NLPHL have excellent long-term survival, identifying patients where treatment de-escalation is appropriate will help to minimize toxicity. De-escalation strategies include observation after fully resected stage I disease, active surveillance, anti-CD20 antibody monotherapy, radiotherapy in early-stage disease, and avoiding anthracycline- or bleomycin-containing chemotherapy regimens. Evidence supporting the use of novel therapies remains limited with disappointing results from a recently published study of ibrutinib in patients with relapsed NLPHL. Hopefully, future trials will investigate novel agents such as checkpoint inhibitors, T-cell engaging antibodies and chimeric antigen receptor T-cell therapy.</p>","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3476-3487"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532684/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142132572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01DOI: 10.3324/haematol.2023.284922
Sonia Jaramillo, Michael Scherer, Chelsea Szu-Tu, Sergi Beneyto-Calabuig, Carsten Müller-Tidow, Richard F Schlenk, Michael Hundemer, Lars Velten, Caroline Pabst
{"title":"Late-onset <i>NPM1</i> mutation in a MYC-amplified relapsed/refractory acute myeloid leukemia patient treated with gemtuzumab ozogamicin and glasdegib.","authors":"Sonia Jaramillo, Michael Scherer, Chelsea Szu-Tu, Sergi Beneyto-Calabuig, Carsten Müller-Tidow, Richard F Schlenk, Michael Hundemer, Lars Velten, Caroline Pabst","doi":"10.3324/haematol.2023.284922","DOIUrl":"10.3324/haematol.2023.284922","url":null,"abstract":"","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":"3811-3815"},"PeriodicalIF":8.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11532717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175173","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}