Izidore S Lossos, Xiaoyu Jiang, Krishna Patel, Kamilia N Moalem, Jordan Laser, Joo Y Song, Anamarija M Perry, Jennifer R Chapman
{"title":"Association Between Moraxella catarrhalis and Nodular Lymphocyte-Predominant Hodgkin Lymphoma.","authors":"Izidore S Lossos, Xiaoyu Jiang, Krishna Patel, Kamilia N Moalem, Jordan Laser, Joo Y Song, Anamarija M Perry, Jennifer R Chapman","doi":"10.1002/ajh.27762","DOIUrl":"10.1002/ajh.27762","url":null,"abstract":"<p><p>Nodular lymphocyte-predominant Hodgkin Lymphoma (NLPHL) may be an antigen-driven malignancy. Recent studies demonstrated that in NLPHL patients, lymphoma B-cell receptor can bind proteins derived from Moraxella catarrhalis (MC) and Rothia mucilaginosa (RM). We examined whether MC and RM can be detected in NLPHL lymph nodes. The presence of MC DNA and RNA was tested by polymerase chain reaction (PCR) and RNAScope in situ hybridization (ISH), respectively. The presence of RM DNA was tested by PCR. We demonstrated the presence of MC DNA in 29 of 49 (59.2%) NLPHL patient tumor samples and RNA in 14 of 39 biopsies (35.9%), with good concordance between the assays, considering lower RNA stability in older biopsies and lower sensitivity of ISH compared to PCR. In comparison, 36 lymph nodes with follicular hyperplasia and a specimen with tubular adenoma were negative for MC by PCR, and only 6 of 37 non-NLPHL lymphoma tissues (16.2%) were positive for detection of MC by PCR, demonstrating a statistically higher detection rate of MC in patients with NLPHL (p = 0.00006). MC was detected in NLPHL samples at diagnosis and relapses, and there was no statistical difference in the detection rate for MC between IgD positive and negative samples. In contrast, RM was detected by PCR in 9 of 49 (18.4%) NLPHL patient tumor samples, always concomitantly with MC without statistical difference in comparison to other lymphomas. The high prevalence of MC in NLPHL tumor tissues provides direct evidence for its association with NLPHL.</p>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":10.1,"publicationDate":"2025-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12258967/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144558834","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}
Fnu Aperna, Maymona G. Abdelmagid, Mahesh Kumar, Joseph H. Butterfield, Thanai Pongdee, Cecilia Y. Arana Yi, Mrinal S. Patnaik, Daniel A. Arber, Attilio Orazi, Dong Chen, Kaaren K. Reichard, Naseema Gangat, Animesh Pardanani, Ayalew Tefferi
{"title":"Systemic Mastocytosis in 910 Patients: Prognostic Contribution of the International Consensus Classification in the Context of the Mayo Alliance Prognostic System","authors":"Fnu Aperna, Maymona G. Abdelmagid, Mahesh Kumar, Joseph H. Butterfield, Thanai Pongdee, Cecilia Y. Arana Yi, Mrinal S. Patnaik, Daniel A. Arber, Attilio Orazi, Dong Chen, Kaaren K. Reichard, Naseema Gangat, Animesh Pardanani, Ayalew Tefferi","doi":"10.1002/ajh.27764","DOIUrl":"https://doi.org/10.1002/ajh.27764","url":null,"abstract":"The current study includes 910 patients with systemic mastocytosis (SM) seen at the Mayo Clinic from 1968 to 2024. The primary objective was to examine the prognostic contribution of the International Consensus Classification (ICC), in the context of the Mayo Alliance Prognostic System (MAPS) for SM. World Health Organization classification (WHO‐HAEM5) subcategories included (i) indolent/smoldering SM (ISM/SSM; <jats:italic>N</jats:italic> = 518), (ii) SM associated with another hematological neoplasm (SM‐AHN; <jats:italic>N</jats:italic> = 273), with the latter including both myeloid and lymphoid neoplasms, (iii) aggressive SM (ASM; <jats:italic>N</jats:italic> = 106), and (iv) WHO‐defined mast cell leukemia (MCL; <jats:italic>N</jats:italic> = 13), which included mast cells with both “mature” and “immature” morphology. The ICC‐defined subcategories were mostly similar with the exception that SM‐AHN was replaced by SM associated with another myeloid neoplasm (SM‐AMN; <jats:italic>N</jats:italic> = 235) and WHO‐defined MCL was replaced by ICC‐defined MCL (<jats:italic>N</jats:italic> = 8), which included only those with immature MC morphology. Overall survival (OS) was similar between WHO‐defined MCL (median 1.8 years) vs. SM‐AHN (median 2.3 years; <jats:italic>p</jats:italic> = 0.3) but significantly different between ICC‐defined MCL (median 0.08 years) vs. SM‐AMN (median 2.0 years; <jats:italic>p</jats:italic> < 0.01). Significant difference in OS was also apparent between ICC‐defined SM‐AMN and SM associated with lymphoid neoplasm (SM‐ALN; median 8.1 years; HR 3.4; <jats:italic>p</jats:italic> < 0.01). Multivariable analysis confirmed the inter‐independent prognostic contributions of both ICC‐defined morphologic subcategories and MAPS risk variables, including age > 60 years, anemia, alkaline phosphatase > ULN, and platelet count < 150 × 10<jats:sup>9</jats:sup>/L (<jats:italic>p</jats:italic> < 0.03 in all instances); the same was not true for WHO‐HAEM5‐defined SM subcategories. The addition of mutational information into the multivariable model resulted in ousting anemia and inclusion of <jats:italic>ASXL1</jats:italic> (<jats:italic>p</jats:italic> < 0.01), <jats:italic>SRSF2</jats:italic> (<jats:italic>p</jats:italic> = 0.01), and <jats:italic>NRAS</jats:italic> (<jats:italic>p</jats:italic> = 0.01) mutations as additional risk factors. Classification of SM by ICC is prognostically more accurate, compared to WHO‐HAEM5, and strengthens the prognostic contribution of morphology to current clinical and molecular risk models.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"105 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144547185","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}
{"title":"Autoimmune Hepatobiliary Disease and Cryoglobulins in Peripheral Blood.","authors":"Anna Shestakova,Anton V Rets,Madhu Menon","doi":"10.1002/ajh.27749","DOIUrl":"https://doi.org/10.1002/ajh.27749","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"91 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144547732","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}
Sara Canovas Nunes,Helen D Reed,Danilo Pellin,Chad Harris,Felicia Andresen,Aya Abu-El-Haija,Christina Barbera,Jaclyn Schienda,Jill A Madden,Jacob R Bledsoe,Karyn Brundige,Megan Day-Lewis,Alan A Nguyen,Akiko Shimamura,David A Williams
{"title":"Regression of Monosomy 7 Clone in Patient With RECQL4-Associated Syndrome.","authors":"Sara Canovas Nunes,Helen D Reed,Danilo Pellin,Chad Harris,Felicia Andresen,Aya Abu-El-Haija,Christina Barbera,Jaclyn Schienda,Jill A Madden,Jacob R Bledsoe,Karyn Brundige,Megan Day-Lewis,Alan A Nguyen,Akiko Shimamura,David A Williams","doi":"10.1002/ajh.27739","DOIUrl":"https://doi.org/10.1002/ajh.27739","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"19 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144533340","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}
Cecelia R. Miller, Ying Huang, John N. Allan, Seema A. Bhat, David Bond, Danielle Brander, John C. Byrd, Julio Chavez, Elise Chong, Alexey Danilov, Mark R. Dowling, Kaitlyn Dvorak‐Kornaus, Paul J. Hampel, Carrie Ho, Prioty Islam, Nikita Malakhov, Matthew Matasar, Sarah Miller, Sameer A. Parikh, Kari G. Rabe, Joanna M. Rhodes, Lindsey E. Roeker, Kerry A. Rogers, Aditi Saha, Alan Skarbnik, Hamish Scott, Jake Schade, Mazyar Shadman, Geoffrey Shouse, Deborah M. Stephens, Meghan C. Thompson, Philip A. Thompson, Yucai Wang, Jennifer A. Woyach, Max Yano, Zulfa Omer, Adam S. Kittai
{"title":"Prognostic Implication of Karyotypic Evaluation in Patients With Richter Transformation in the Modern Era","authors":"Cecelia R. Miller, Ying Huang, John N. Allan, Seema A. Bhat, David Bond, Danielle Brander, John C. Byrd, Julio Chavez, Elise Chong, Alexey Danilov, Mark R. Dowling, Kaitlyn Dvorak‐Kornaus, Paul J. Hampel, Carrie Ho, Prioty Islam, Nikita Malakhov, Matthew Matasar, Sarah Miller, Sameer A. Parikh, Kari G. Rabe, Joanna M. Rhodes, Lindsey E. Roeker, Kerry A. Rogers, Aditi Saha, Alan Skarbnik, Hamish Scott, Jake Schade, Mazyar Shadman, Geoffrey Shouse, Deborah M. Stephens, Meghan C. Thompson, Philip A. Thompson, Yucai Wang, Jennifer A. Woyach, Max Yano, Zulfa Omer, Adam S. Kittai","doi":"10.1002/ajh.27760","DOIUrl":"https://doi.org/10.1002/ajh.27760","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"27 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532939","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}
Hanny Al‐Samkari, Tracy J. Mayne, Misty Troutt, Hemant Patle, Marianne Clancy, Eric Duhaime
{"title":"Characterizing the Healthcare Utilization and Costs of Hereditary Hemorrhagic Telangiectasia","authors":"Hanny Al‐Samkari, Tracy J. Mayne, Misty Troutt, Hemant Patle, Marianne Clancy, Eric Duhaime","doi":"10.1002/ajh.27756","DOIUrl":"https://doi.org/10.1002/ajh.27756","url":null,"abstract":"Hereditary hemorrhagic telangiectasia (HHT) is the second‐most common inherited bleeding disorder worldwide, afflicting one in 4000–5000 people, and is the most morbid inherited bleeding disorder of women. HHT causes recurrent severe epistaxis, chronic gastrointestinal bleeding, heavy menstrual bleeding, and arteriovenous malformations in the lung, liver, and brain that cause serious bleeding and nonbleeding complications. There are no approved treatments worldwide, and the direct medical costs of HHT have not been well‐characterized. We utilized the Komodo Healthcare Map claims database to create a large sample of US patients diagnosed with HHT, including a subgroup with anemia and a subgroup receiving hematologic support (iron infusions and/or red cell transfusions). We quantified mean per patient per year (PPPY) and total population inpatient, outpatient, and pharmacy costs in 2022 and 2023. The mean PPPY costs for all HHT patients (<jats:italic>n</jats:italic> = 24 407; <jats:italic>n</jats:italic> = 23 524), those with anemia (<jats:italic>n</jats:italic> = 13 856; <jats:italic>n</jats:italic> = 13 192) and those receiving hematologic support (<jats:italic>n</jats:italic> = 6191; <jats:italic>n</jats:italic> = 5818) were approximately $19 000, $27 000, and $40 000, respectively, across years, representing > $450 000 000 in annual healthcare costs in the sample. The leading cost drivers were related to treatment for bleeding and its consequences. While accounting for nearly 60% of HHT patients, those with anemia accounted for ~80% of direct medical costs. Across the majority of leading inpatient, outpatient, and pharmacy cost drivers, patients with anemia and anemia treatment accounted for 75%–100% of cost. The PPPY costs of HHT are comparable to, or exceed, those of other rare, resource‐intensive serious diseases, including sickle cell disease, muscular dystrophy, and cystic fibrosis.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"19 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144533088","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}
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