keyword
https://read.qxmd.com/read/38597584/predictors-of-clinical-outcome-in-myeloproliferative-neoplasm-unclassifiable-a-bone-marrow-pathology-group-study
#1
JOURNAL ARTICLE
Genevieve M Crane, Julia T Geyer, Beenu Thakral, Sa A Wang, Geoffrey D Wool, Ke David Li, Adam R Davis, Leonardo Boiocchi, David Bosler, Carlos E Bueso-Ramos, Daniel A Arber, Tracy I George, Adam Bagg, Robert P Hasserjian, Attilio Orazi, Eric D Hsi, Heesun J Rogers
OBJECTIVES: Myeloproliferative neoplasm, unclassifiable (MPN-U, revised to MPN, not otherwise specified in the fifth edition of the World Health Organization classification) is a heterogeneous category of primary marrow disorders with clinical, morphologic, and/or molecular features that preclude classification as a more specific MPN subtype due to stage at diagnosis, overlapping features between MPN subtypes, or the presence of coexisting disorders. Compared with other MPN subtypes, the contribution of the mutational landscape in MPN-U in conjunction with other clinical and morphologic biomarkers to prognosis has been less well investigated...
April 10, 2024: American Journal of Clinical Pathology
https://read.qxmd.com/read/38290600/follicular-lymphoma-presenting-with-symptomatic-bone-involvement-a-clinicopathologic-and-molecular-analysis-of-16-cases
#2
JOURNAL ARTICLE
Rossella Sarro, Bettina Bisig, Baptiste Guey, Edoardo Missiaglia, Anne Cairoli, Patrick Omoumi, Igor Letovanec, Judith A Ferry, Robert P Hasserjian, Laurence de Leval
Primary bone lymphoma (PBL) is rare and mostly represented by diffuse large B-cell lymphomas (DLBCL). Follicular lymphoma (FL), albeit commonly disseminating to the bone marrow, rarely presents primarily as bone lesions. Here, we studied 16 patients (12 men:4 women, median age 60 years) who presented with bone pain and/or skeletal radiologic abnormalities revealing bone FL. Lesions were multifocal in 11 patients (spine ± appendicular skeleton), and unifocal in 5 patients (femoral, tibial, or vertebral)...
January 28, 2024: Modern Pathology
https://read.qxmd.com/read/37981812/-stat5b-mutations-in-myeloid-neoplasms-differ-by-disease-subtypes-but-characterize-a-subset-of-chronic-myeloid-neoplasms-with-eosinophilia-and-or-basophilia
#3
JOURNAL ARTICLE
C Cameron Yin, Wayne Tam, Serena M Walker, Amandeep Kaur, Madhu M Ouseph, Wei Xie, Olga K Weinberg, Peng Li, Zhuang Zuo, Mark J Routbort, Simon Chen, L Jeffrey Medeiros, Tracy I George, Attilio Orazi, Daniel A Arber, Adam Bagg, Robert P Hasserjian, Sa A Wang
STAT5B has been reported as a recurrent mutation in myeloid neoplasms (MNs) with eosinophilia, but the overall frequency and importance across a spectrum of MNs are largely unknown. We conducted a multicenter study on a series of 82 MNs with STAT5B mutations detected by next-generation sequencing. The estimated frequency of STAT5B mutation in MNs was low.
November 16, 2023: Haematologica
https://read.qxmd.com/read/37865504/morphologic-characteristics-of-myelodysplastic-syndromes
#4
REVIEW
Lisa D Yuen, Robert P Hasserjian
Morphologic characterization remains a cornerstone in the diagnosis and classification of myelodysplastic syndromes (MDS) in the updated International Consensus Classification (ICC) and 5th edition World Health Organization Classification of Myeloid Neoplasms (Arber, Orazi, & Hasserjian, 2022; Khoury & Solary, 2022). The presence of dysplasia is one of the key diagnostic criteria required for establishing a diagnosis of MDS, and the percentage of myeloblasts in the blood and bone marrow impacts both disease classification and prognostication...
December 2023: Clinics in Laboratory Medicine
https://read.qxmd.com/read/37839675/cutaneous-manifestations-of-myeloid-neoplasms-exhibit-broad-and-divergent-morphologic-and-immunophenotypic-features-but-share-ancestral-clonal-mutations-with-bone-marrow
#5
JOURNAL ARTICLE
Sam Sadigh, Daniel J DeAngelo, Jacqueline S Garcia, Robert P Hasserjian, Christopher B Hergott, Andrew A Lane, Scott B Lovitch, Fabienne Lucas, Marlise R Luskin, Elizabeth A Morgan, Geraldine S Pinkus, Olga Pozdnyakova, Scott J Rodig, Vignesh Shanmugam, Harrison K Tsai, Eric S Winer, David Zemmour, Annette S Kim
In this study, we performed a comprehensive molecular analysis of paired skin and peripheral blood/bone marrow (BM) samples from 17 patients with cutaneous myeloid or cutaneous histiocytic-dendritic neoplasms. The cutaneous manifestations included 10 patients with cutaneous acute myeloid leukemia (c-AML), 2 patients with full or partial Langerhans cell differentiation, 2 patients with blastic plasmacytoid dendritic cell neoplasms (BPDCN), 1 patient with both Langerhans cell differentiation and BPDCN, and 2 patients with full or partial indeterminate dendritic cell differentiation...
October 13, 2023: Modern Pathology
https://read.qxmd.com/read/37774372/diagnosis-and-classification-of-myelodysplastic-syndromes
#6
COMMENT
Robert P Hasserjian, Ulrich Germing, Luca Malcovati
Myelodysplastic syndromes (MDSs) are neoplastic myeloid proliferations characterized by ineffective hematopoiesis resulting in peripheral blood cytopenias. MDS is distinguished from nonneoplastic clonal myeloid proliferations by the presence of morphologic dysplasia and from acute myeloid leukemia by a blast threshold of 20%. The diagnosis of MDS can be challenging because of the myriad other causes of cytopenias: accurate diagnosis requires the integration of clinical features with bone marrow and peripheral blood morphology, immunophenotyping, and genetic testing...
December 28, 2023: Blood
https://read.qxmd.com/read/37733312/case-29-2023-a-34-year-old-woman-with-abdominal-distention-and-acute-kidney-injury
#7
JOURNAL ARTICLE
Sundar Jagannath, Mark A Anderson, Andrew J Yee, Robert P Hasserjian
New England Journal of Medicine, Volume 389, Issue 12, Page 1133-1143, September 2023.
September 21, 2023: New England Journal of Medicine
https://read.qxmd.com/read/37704469/classification-risk-stratification-and-response-assessment-in-myelodysplastic-syndromes-neoplasms-mds-a-state-of-the-art-report-on-behalf-of-the-international-consortium-for-mds-icmds
#8
REVIEW
Maximilian Stahl, Jan Philipp Bewersdorf, Zhuoer Xie, Matteo Giovanni Della Porta, Rami Komrokji, Mina L Xu, Omar Abdel-Wahab, Justin Taylor, David P Steensma, Daniel T Starczynowski, Mikkael A Sekeres, Guillermo Sanz, David A Sallman, Gail J Roboz, Uwe Platzbecker, Mrinal M Patnaik, Eric Padron, Olatoyosi Odenike, Stephen D Nimer, Aziz Nazha, Ravi Majeti, Sanam Loghavi, Richard F Little, Alan F List, Tae Kon Kim, Christopher S Hourigan, Robert P Hasserjian, Stephanie Halene, Elizabeth A Griffiths, Steven D Gore, Peter Greenberg, Maria E Figueroa, Pierre Fenaux, Fabio Efficace, Amy E DeZern, Naval G Daver, Jane E Churpek, Hetty E Carraway, Rena Buckstein, Andrew M Brunner, Jacqueline Boultwood, Uma Borate, Rafael Bejar, John M Bennett, Andrew H Wei, Valeria Santini, Michael R Savona, Amer M Zeidan
The guidelines for classification, prognostication, and response assessment of myelodysplastic syndromes/neoplasms (MDS) have all recently been updated. In this report on behalf of the International Consortium for MDS (icMDS) we summarize these developments. We first critically examine the updated World Health Organization (WHO) classification and the International Consensus Classification (ICC) of MDS. We then compare traditional and molecularly based risk MDS risk assessment tools. Lastly, we discuss limitations of criteria in measuring therapeutic benefit and highlight how the International Working Group (IWG) 2018 and 2023 response criteria addressed these deficiencies and are endorsed by the icMDS...
November 2023: Blood Reviews
https://read.qxmd.com/read/37601841/presence-but-not-number-of-secondary-type-mutations-influences-outcome-in-de-novo-aml-without-mds-associated-or-recurring-cytogenetic-abnormalities
#9
JOURNAL ARTICLE
Olga K Weinberg, Miguel D Cantu, Jeffrey Gagan, Yazan F Madanat, Daniel A Arber, Robert P Hasserjian
A group of gene mutations has been identified to be strongly associated with secondary acute myeloid leukemias (AML) arising from prior myeloid neoplasms. The International Consensus Classification (ICC) and proposed 5th edition of the World Health Organization (WHO) classification differ by inclusion of RUNX1 . A recent study suggested that having two or more secondary mutations is associated with a particularly poor prognosis. In a study of 294 de novo AML patients, we found that patients with at least one ICC-defined secondary mutation had shorter survival when compared to those without secondary mutations, and ICC/WHO groups of two or more mutations did not predict for worse outcomes...
August 2023: EJHaem
https://read.qxmd.com/read/37524919/prognostic-impact-of-secondary-versus-de-novo-ontogeny-in-acute-myeloid-leukemia-is-accounted-for-by-the-european-leukemianet-2022-risk-classification
#10
MULTICENTER STUDY
Michael J Hochman, Megan Othus, Robert P Hasserjian, Alex Ambinder, Andrew Brunner, Mary-Elizabeth M Percival, Christopher S Hourigan, Ronan Swords, Amy E DeZern, Elihu H Estey, Judith E Karp
Secondary AML (sAML), defined by either history of antecedent hematologic disease (AHD) or prior genotoxic therapy (tAML), is classically regarded as having worse prognosis than de novo disease (dnAML). Clinicians may infer a new AML diagnosis is secondary based on a history of antecedent blood count (ABC) abnormalities in the absence of known prior AHD, but whether abnormal ABCs are associated with worse outcomes is unclear. Secondary-type mutations have recently been incorporated into the European LeukemiaNet (ELN) 2022 guidelines as adverse-risk features, raising the question of whether clinical descriptors of ontogeny (i...
September 2023: Leukemia
https://read.qxmd.com/read/37391178/guide-to-the-diagnosis-of-myeloid-neoplasms-a-bone-marrow-pathology-group-approach
#11
JOURNAL ARTICLE
Kathryn Foucar, Adam Bagg, Carlos E Bueso-Ramos, Tracy George, Robert P Hasserjian, Eric D Hsi, Attilio Orazi, Wayne Tam, Sa A Wang, Olga K Weinberg, Daniel A Arber
OBJECTIVES: The practicing pathologist is challenged by the ever-increasing diagnostic complexity of myeloid neoplasms. This guide is intended to provide a general roadmap from initial case detection, often triggered by complete blood count results with subsequent blood smear review, to final diagnosis. METHODS: The integration of hematologic, morphologic, immunophenotypic, and genetic features into routine practice is standard of care. The requirement for molecular genetic testing has increased along with the complexity of test types, the utility of different testing modalities in identifying key gene mutations, and the sensitivity and turnaround time for various assays...
October 3, 2023: American Journal of Clinical Pathology
https://read.qxmd.com/read/37283522/the-international-consensus-classification-of-eosinophilic-disorders-and-systemic-mastocytosis
#12
REVIEW
Sa A Wang, Attilio Orazi, Jason Gotlib, Andreas Reiter, Alexandar Tzankov, Robert P Hasserjian, Daniel A Arber, Ayalew Tefferi
Based on new data and increased understanding of disease molecular genetics, the international consensus classification (ICC) has made several changes in the diagnosis and classification of eosinophilic disorders and systemic mastocytosis. Myeloid/lymphoid neoplasms with eosinophilia (M/LN-eo) and gene rearrangements have been renamed as M/LN-eo with tyrosine kinase gene fusions (M/LN-eo-TK). The category has been expanded to include ETV6::ABL1 and FLT3 fusions, and to accept PCM1::JAK2 and its genetic variants as formal members...
August 2023: American Journal of Hematology
https://read.qxmd.com/read/37210875/acute-leukemias-with-complex-karyotype-show-a-similarly-poor-outcome-independent-of-mixed-myeloid-or-lymphoblastic-immunophenotype-a-study-from-the-bone-marrow-pathology-group
#13
JOURNAL ARTICLE
Timothy Kirtek, Weina Chen, Dorottya Laczko, Adam Bagg, Prasad Koduru, Kathryn Foucar, Elise Venable, Meredith Nichols, Heesun J Rogers, Wayne Tam, Attilio Orazi, Eric D Hsi, Robert P Hasserjian, Sa A Wang, Daniel A Arber, Olga K Weinberg
Mixed phenotype acute leukemia (MPAL) is a heterogenous group of acute leukemias characterized by leukemic blasts that express markers of multiple lineages. The revised 4th edition WHO classification of MPAL excludes AML with myelodysplasia related changes (AML-MRC), including those with complex karyotype (CK), from a diagnosis of MPAL. Abnormal karyotype is frequent in MPAL with the reported rate of CK in MPAL ranging from 19% to 32%. Due its rarity, the clinical and genetic features of MPAL with CK remain poorly characterized...
May 10, 2023: Leukemia Research
https://read.qxmd.com/read/37195767/updates-in-classification-of-myelodysplastic-syndrome
#14
REVIEW
Mina L Xu, Robert P Hasserjian
Myelodysplastic syndrome includes a broad range of myeloid neoplasms characterized by cytopenia and morphologic dysplasia. Recently, 2 new classification systems emerged to further define how these diseases are diagnosed and risk stratified. This review compares these models, provides detailed approaches, and reveals practical ways to move forward in clinical practice of myelodysplastic syndrome diagnosis.
May 2023: Cancer Journal
https://read.qxmd.com/read/37173164/pediatric-myelodysplastic-syndrome
#15
REVIEW
Stephen Babcock, Katherine R Calvo, Robert P Hasserjian
No abstract text is available yet for this article.
May 2023: Seminars in Diagnostic Pathology
https://read.qxmd.com/read/37165840/safety-and-efficacy-of-zinpentraxin-alfa-as-monotherapy-or-in-combination-with-ruxolitinib-in-myelofibrosis-stage-i-of-a-phase-ii-trial
#16
JOURNAL ARTICLE
Srdan Verstovsek, Lynda Foltz, Vikas Gupta, Robert Hasserjian, Taghi Manshouri, John Mascarenhas, Ruben Mesa, Olga Pozdnyakova, Ellen Ritchie, Ivo Veletic, Katia Gamel, Habib Hamidi, Lyrialle Han, Brian Higgins, Kerstin Trunzer, Marianne Uguen, Dao Wang, Tarec Christoffer El-Galaly, Boyan Todorov, Jason Gotlib
Pentraxin 2 (PTX-2; serum amyloid P component), a circulating endogenous regulator of the inflammatory response to tissue injury and fibrosis, is reduced in patients with myelofibrosis (MF). Zinpentraxin alfa (RO7490677, PRM-151) is a recombinant form of PTX-2 that has shown preclinical antifibrotic activity and no dose-limiting toxicities in phase I trials. We report results from stage 1 of a phase II trial of zinpentraxin alfa in patients with intermediate-1/2 or high-risk MF. Patients (n=27) received intravenous zinpentraxin alfa weekly (QW) or every 4 weeks (Q4W), as monotherapy or an additional therapy for patients on stable-dose ruxolitinib...
May 11, 2023: Haematologica
https://read.qxmd.com/read/37146262/computer-vision-quantitation-of-erythrocyte-shape-abnormalities-provides-diagnostic-prognostic-and-mechanistic-insight
#17
JOURNAL ARTICLE
Brody Foy, Jonathan Stefely, Pavan K Bendapudi, Robert P Hasserjian, Hanny Al-Samkari, Abner Louissaint, Megan Fitzpatrick, Bailey Hutchison, Christopher Mow, Julia Collins, Hasmukh Patel, Chhaya Patel, Nikita Patel, Samantha Ho, Richard M Kaufman, Walter 'Sunny' Dzik, John M Higgins, Robert S Makar
Examination of red blood cell (RBC) morphology in peripheral blood smears can help diagnose hematologic disease, even in resource-limited settings, but this analysis remains subjective and semi-quantitative with low throughput. Prior attempts to develop automated tools have been hampered by poor reproducibility and limited clinical validation. Here, we present a novel, open-source machine-learning approach (denoted the 'RBC-diff') to quantify abnormal RBCs in peripheral smear images and generate an RBC morphology differential...
May 5, 2023: Blood Advances
https://read.qxmd.com/read/36960680/myeloid-sarcoma-with-npm1-mutation-may-be-clinically-and-genetically-distinct-from-aml-with-npm1-mutation-a-study-from-the-bone-marrow-pathology-group
#18
JOURNAL ARTICLE
Maximiliano Ramia de Cap, Leo P Wu, Christian Hirt, German A Pihan, Sanjay S Patel, Wayne Tam, Carlos E Bueso-Ramos, Rashmi Kanagal-Shamanna, Philipp W Raess, Alexa Siddon, Damodaran Narayanan, Elizabeth A Morgan, Geraldine S Pinkus, Emily F Mason, Eric D Hsi, Heesun J Rogers, Laura Toth, Kathryn Foucar, Stephanie N Hurwitz, Adam Bagg, Anton Rets, Tracy I George, Attilio Orazi, Daniel A Arber, Robert P Hasserjian, Olga K Weinberg
Myeloid sarcoma (MS) is currently considered equivalent to de novo acute myeloid leukemia (AML); however, the relationship between these entities is poorly understood. This retrospective multi-institutional cohort study compared 43 MS with NPM1 mutation to 106 AML with NPM1 mutation. Compared to AML, MS had more frequent cytogenetic abnormalities including complex karyotype ( p  = .009 and p  = .007, respectively) and was enriched in mutations of genes involved in histone modification, including ASXL1 ( p  = ...
March 24, 2023: Leukemia & Lymphoma
https://read.qxmd.com/read/36934059/current-landscape-of-translational-and-clinical-research-in-myelodysplastic-syndromes-neoplasms-mds-proceedings-from-the-1-st-international-workshop-on-mds-iwmds-of-the-international-consortium-for-mds-icmds
#19
REVIEW
Jan Philipp Bewersdorf, Zhuoer Xie, Rafael Bejar, Uma Borate, Jacqueline Boultwood, Andrew M Brunner, Rena Buckstein, Hetty E Carraway, Jane E Churpek, Naval G Daver, Matteo Giovanni Della Porta, Amy E DeZern, Pierre Fenaux, Maria E Figueroa, Steven D Gore, Elizabeth A Griffiths, Stephanie Halene, Robert P Hasserjian, Christopher S Hourigan, Tae Kon Kim, Rami Komrokji, Vijay K Kuchroo, Alan F List, Sanam Loghavi, Ravindra Majeti, Olatoyosi Odenike, Mrinal M Patnaik, Uwe Platzbecker, Gail J Roboz, David A Sallman, Valeria Santini, Guillermo Sanz, Mikkael A Sekeres, Maximilian Stahl, Daniel T Starczynowski, David P Steensma, Justin Taylor, Omar Abdel-Wahab, Mina L Xu, Michael R Savona, Andrew H Wei, Amer M Zeidan
Biological events that contribute to the pathogenesis of myelodysplastic syndromes/neoplasms (MDS) are becoming increasingly characterized and are being translated into rationally designed therapeutic strategies. Herein, we provide updates from the first International Workshop on MDS (iwMDS) of the International Consortium for MDS (icMDS) detailing recent advances in understanding the genetic landscape of MDS, including germline predisposition, epigenetic and immune dysregulation, the complexities of clonal hematopoiesis progression to MDS, as well as novel animal models of the disease...
July 2023: Blood Reviews
https://read.qxmd.com/read/36788093/triple-negative-primary-myelofibrosis-a-bone-marrow-pathology-group-study
#20
JOURNAL ARTICLE
Yahya A Al-Ghamdi, Jonathan Lake, Adam Bagg, Beenu Thakral, Sa A Wang, Carlos Bueso-Ramos, Lucia Masarova, Srdan Verstovsek, Heesun J Rogers, Eric D Hsi, Jonathon H Gralewski, Devon Chabot-Richards, Tracy I George, Anton Rets, Robert P Hasserjian, Olga K Weinberg, Megan Parilla, Daniel A Arber, Osvaldo Padilla, Attilio Orazi, Wayne Tam
Primary myelofibrosis (PMF) is a clonal myeloproliferative neoplasm driven by canonical gene mutations in JAK2, CALR, or MPL in >80% of the cases. PMF that lacks these canonical alterations is termed triple-negative PMF (TN-PMF). The pathologic and genetic characteristics of TN-PMF compared with those of conventional PMF with canonical driver mutations (DM-PMF) have not been well studied. We aimed to identify clinicopathologic and molecular genetic differences between patients with TN-PMF (n = 56) and DM-PMF (n = 89), all of whom fulfilled the 2016 World Health Organization diagnostic criteria for PMF...
January 10, 2023: Modern Pathology
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