Journal of hematology最新文献

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Unraveling the Rare Entity of KIT D816V-Negative Systemic Mastocytosis. 揭开 KIT D816V 阴性系统性肥大细胞增多症罕见实体的神秘面纱
IF 1.3
Journal of hematology Pub Date : 2024-06-01 Epub Date: 2024-06-28 DOI: 10.14740/jh1279
Ruah Alyamany, Chams Alkhalaf Albachir, Sarah Alsaleh, Alaa Hamad, Sameeha Kaiser Abdulwali, Ahmad S Alotaibi, Syed Osman Ahmed, Mansour Alfayez
{"title":"Unraveling the Rare Entity of <i>KIT</i> D816V-Negative Systemic Mastocytosis.","authors":"Ruah Alyamany, Chams Alkhalaf Albachir, Sarah Alsaleh, Alaa Hamad, Sameeha Kaiser Abdulwali, Ahmad S Alotaibi, Syed Osman Ahmed, Mansour Alfayez","doi":"10.14740/jh1279","DOIUrl":"10.14740/jh1279","url":null,"abstract":"<p><p>Systemic mastocytosis (SM) is a rare type of myeloproliferative neoplasm characterized by abnormal proliferation and infiltration of different tissue by clonal mast cells. The uncontrolled proliferation and activation of mast cells trigger the release of vasoactive and inflammatory mediators, resulting in a cascade of systemic symptoms. Around 95% of SM arise from a gain-of-function mutation at the <i>KIT</i> gene, specifically at codon 816, which highlights its essential role in SM and makes it an attractive target for therapy. Although <i>KIT</i>-negative SM is exceptionally rare, the increased number of cases documented in the literature makes it an intriguing dimension of this disorder. The reported clinical manifestations of <i>KIT</i>-negative SM are widely variable, but many are similar to <i>KIT</i>-positive SM. KIT-targeted therapeutic options have been a game-changer in <i>KIT</i>-positive SM, however their role in <i>KIT</i>-negative SM remains controversial. This report aimed to further understand <i>KIT</i>-negative SM by presenting two cases of <i>KIT</i>-negative SM, one of which was responsive to KIT-targeted therapy, and analyzing reported cases in the existing literature.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 3","pages":"128-136"},"PeriodicalIF":1.3,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11236357/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Unique Case of a Compound Heterozygosity of Hemoglobin Korle-Bu and Sickle Cell Trait in a Military Trainee. 一个军校学员血红蛋白 Korle-Bu 和镰状细胞特质复合杂合子的独特病例。
IF 1.3
Journal of hematology Pub Date : 2024-06-01 Epub Date: 2024-06-28 DOI: 10.14740/jh1257
Gartrell C Bowling, Niels A Ryden, Allen R Holmes, Lauren E Lee, Kristin Stoll
{"title":"A Unique Case of a Compound Heterozygosity of Hemoglobin Korle-Bu and Sickle Cell Trait in a Military Trainee.","authors":"Gartrell C Bowling, Niels A Ryden, Allen R Holmes, Lauren E Lee, Kristin Stoll","doi":"10.14740/jh1257","DOIUrl":"10.14740/jh1257","url":null,"abstract":"<p><p>Hemoglobin Korle-Bu (Hb KB) is a rare and likely under-reported hemoglobin (Hb) variant resulting from an unusual point mutation on the beta-globin chain. Hb KB is typically clinically silent, and there are limited reports of Hb KB heterozygosity compounded with other hemoglobinopathies that can present with varying clinical phenotypes. Here, we report a case of compound Hb KB heterozygosity with Hb S in an asymptomatic military trainee with a positive sickle cell screening test. Hb capillary and gel electrophoresis predicted a compound Hb S/D-Punjab overlap, which foretells a severe clinical phenotype. Sequencing of the Hb beta gene <i>HBB</i> demonstrated Hb KB, allowing for a diagnosis that fit his asymptomatic clinical phenotype and allowed for retention in the military.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 3","pages":"116-120"},"PeriodicalIF":1.3,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11236362/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Predictors of Non-Variceal Hemorrhage in a National Cohort of Patients With Chronic Liver Disease. 全国慢性肝病患者队列中的非静脉出血预测因素。
IF 1.3
Journal of hematology Pub Date : 2024-06-01 Epub Date: 2024-06-28 DOI: 10.14740/jh1214
Amber Afzal, Preethi Kesavan, Luo Suhong, Brian F Gage, Kevin Korenblat, Martin Schoen, Kristen Sanfilippo
{"title":"Predictors of Non-Variceal Hemorrhage in a National Cohort of Patients With Chronic Liver Disease.","authors":"Amber Afzal, Preethi Kesavan, Luo Suhong, Brian F Gage, Kevin Korenblat, Martin Schoen, Kristen Sanfilippo","doi":"10.14740/jh1214","DOIUrl":"10.14740/jh1214","url":null,"abstract":"<p><strong>Background: </strong>Non-variceal hemorrhage in patients with chronic liver disease (CLD) increases morbidity, mortality, and healthcare costs. There are limited data on risk factors for non-variceal hemorrhage in the CLD population. The aim of this study was to assess the predictive value of various clinical and laboratory parameters for non-variceal hemorrhage in CLD patients.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study of US veterans diagnosed with CLD between 2002 and 2018 within the Veterans Health Administration database. We derived candidate variables from existing risk prediction models for hemorrhage, risk calculators for severity of liver disease, Charlson index of prognostic comorbidities, and prior literature. We used a competing risk analysis to study the relationship between putative risk factors and incidence of non-variceal hemorrhage in patients with CLD.</p><p><strong>Results: </strong>Of 15,183 CLD patients with no history of cancer or anticoagulation use, 674 experienced non-variceal hemorrhage within 1 year of CLD diagnosis. In multivariable analysis, 11 of the 26 candidate variables independently predicted non-variceal hemorrhage: race, international normalized ratio (INR) > 1.5, bilirubin ≥ 2 mg/dL, albumin ≤ 3.5 g/dL, anemia, alcohol abuse, antiplatelet therapy, chronic kidney disease, dementia, proton pump inhibitor prescription, and recent infection.</p><p><strong>Conclusions: </strong>In this study of almost 15,000 veterans, risk factors for non-variceal bleeding within the first year after diagnosis of CLD included non-Caucasian race, laboratory parameters indicating severe liver disease and recent infection in addition to the risk factors for bleeding observed in a general non-CLD population.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 3","pages":"71-78"},"PeriodicalIF":1.3,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11236355/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Steady-State Versus Chemotherapy-Based Stem Cell Mobilization in Multiple Myeloma: A Single-Center Study to Analyze Efficacy and Safety. 多发性骨髓瘤干细胞动员的稳态疗法与化疗疗法:分析疗效和安全性的单中心研究。
IF 1.3
Journal of hematology Pub Date : 2024-06-01 Epub Date: 2024-06-28 DOI: 10.14740/jh1256
Nora Obajed Al-Ali, Laszlo Imre Pinczes, Katalin Farkas, Gyorgy Kerekes, Arpad Illes, Laszlo Varoczy
{"title":"Steady-State Versus Chemotherapy-Based Stem Cell Mobilization in Multiple Myeloma: A Single-Center Study to Analyze Efficacy and Safety.","authors":"Nora Obajed Al-Ali, Laszlo Imre Pinczes, Katalin Farkas, Gyorgy Kerekes, Arpad Illes, Laszlo Varoczy","doi":"10.14740/jh1256","DOIUrl":"10.14740/jh1256","url":null,"abstract":"<p><strong>Background: </strong>High-dose chemotherapy followed by autologous hematopoietic stem cell support is recommended in the treatment of eligible multiple myeloma (MM) patients. The aim of this study was to compare the efficacy and safety of steady-state versus chemotherapy-based stem cell mobilization in our Hungarian patient population.</p><p><strong>Methods: </strong>The subjects were 210 MM patients who underwent stem cell mobilization procedure between 2018 and 2022. Solo granulocyte colony-stimulating factor (G-CSF) was administered in 104 cases, while 106 patients received chemotherapy which was followed by G-CSF administration. We evaluated the ratio of successful mobilizations, the amount of collected stem cells, the incidence of infections and cost-effectivity in the two groups.</p><p><strong>Results: </strong>In the steady-state group, there was a significantly higher need for plerixafor (45% vs. 13%, P < 0.001), unsuccessful stem cell mobilization was more frequent (11% vs. 3%, P = 0.024) and the mean amount of collected stem cells was lower (6.9 vs. 9.8 × 10<sup>6</sup>, P < 0.001) than in the chemotherapy group. However, infections were less frequent (4% vs. 27%, P < 0.001) and the number of days spent in hospital was significantly lower (6 vs. 14 days, P < 0.001). Plerixafor was more frequently administered in those who had received lenalidomide or daratumumab than in those who had been treated with other regimens (41% vs. 23%, P = 0.007 and 78% vs. 23%, P < 0.001, respectively).</p><p><strong>Conclusions: </strong>Steady-state mobilization is a safe method; however, the higher rate of plerixafor administration and unsuccessful attempts may question its superiority to chemomobilization.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 3","pages":"79-85"},"PeriodicalIF":1.3,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11236354/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Middle Meningeal Artery Embolization in Acute Leukemia Patients Presenting With Subdural Hematoma. 急性白血病患者出现硬膜下血肿时的中脑膜动脉栓塞术
IF 1.2
Journal of hematology Pub Date : 2024-04-01 Epub Date: 2024-01-10 DOI: 10.14740/jh1215
Binoy Yohannan, Juan Carlos Martinez Gutierrez, Peng Roc Chen, Adan Rios
{"title":"Middle Meningeal Artery Embolization in Acute Leukemia Patients Presenting With Subdural Hematoma.","authors":"Binoy Yohannan, Juan Carlos Martinez Gutierrez, Peng Roc Chen, Adan Rios","doi":"10.14740/jh1215","DOIUrl":"https://doi.org/10.14740/jh1215","url":null,"abstract":"<p><p>Intracerebral hemorrhage is a potentially fatal complication in patients with acute leukemia and contributing factors include thrombocytopenia and coagulopathy. Patients with acute leukemia may develop subdural hematoma (SDH) spontaneously or secondary to trauma. In patients with acute leukemia and SDH, the surgical evacuation of the hematoma causes significant morbidity and mortality. New approaches and strategies to reduce the need for surgical evacuation are needed to improve outcomes in patients with acute leukemia and intracerebral hemorrhage. We report two cases of acute SDH in patients with acute leukemia successfully treated with middle meningeal artery embolization, a minimally invasive interventional radiology technique, obviating the need for a surgical intervention. The first patient with acute promyelocytic leukemia (APL) presented with coagulopathy and developed an acute SDH after a fall. The second patient with acute myeloid leukemia presented with gum bleeding and also sustained an acute SDH after a fall. Both patients underwent middle meningeal artery embolization for treating their SDHs while actively receiving induction chemotherapy for acute leukemia. Both patients had resolution of their acute SDH and are in remission from their acute leukemia. Middle meningeal artery embolization is a very effective, and within the context of this setting, a novel, minimally invasive technique for management of SDH in acute leukemia patients, which can prevent the need for surgical interventions with its associated comorbidities and high risk of fatal outcomes in patients with acute leukemia and acute SDH.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 1-2","pages":"39-45"},"PeriodicalIF":1.2,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11027772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140856468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Extensive Intracardiac Cement Embolism in a Patient Undergoing Workup for Bone Marrow Transplant 一名正在接受骨髓移植检查的患者发生大面积心内水泥栓塞
IF 1.2
Journal of hematology Pub Date : 2023-12-01 DOI: 10.14740/jh1202
Gavisha Waidyaratne, Caitlin Bennett, Elvira Umyarova, N. Bumma
{"title":"Extensive Intracardiac Cement Embolism in a Patient Undergoing Workup for Bone Marrow Transplant","authors":"Gavisha Waidyaratne, Caitlin Bennett, Elvira Umyarova, N. Bumma","doi":"10.14740/jh1202","DOIUrl":"https://doi.org/10.14740/jh1202","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"55 1","pages":""},"PeriodicalIF":1.2,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139187787","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of Cytogenetic Abnormalities, Induction and Maintenance Regimens on Outcomes After High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients With Newly Diagnosed Multiple Myeloma: A Decade-Long Real-World Experience 细胞遗传学异常、诱导和维持治疗方案对新诊断多发性骨髓瘤患者大剂量化疗和自体干细胞移植后疗效的影响:长达十年的真实世界经验
IF 1.2
Journal of hematology Pub Date : 2023-12-01 DOI: 10.14740/jh1201
A. Thurlapati, William Wesson, James A. Davis, Kelly J. Gaffney, Erin Weeda, Arash Velayati, Jonathan K Bakos, Katelynn Granger, Deidra Smith, Andy Maldonado, Taylor Herrington, Julia Potts, Hamza Hashmi
{"title":"Impact of Cytogenetic Abnormalities, Induction and Maintenance Regimens on Outcomes After High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients With Newly Diagnosed Multiple Myeloma: A Decade-Long Real-World Experience","authors":"A. Thurlapati, William Wesson, James A. Davis, Kelly J. Gaffney, Erin Weeda, Arash Velayati, Jonathan K Bakos, Katelynn Granger, Deidra Smith, Andy Maldonado, Taylor Herrington, Julia Potts, Hamza Hashmi","doi":"10.14740/jh1201","DOIUrl":"https://doi.org/10.14740/jh1201","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 4","pages":""},"PeriodicalIF":1.2,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139187882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful Ultrasound-Guided Spinal Anesthesia in a Patient With Severe Hemophilia A Undergoing Total Hip Arthroplasty 超声引导脊柱麻醉在一名接受全髋关节置换术的重度血友病 A 患者身上的成功应用
IF 1.2
Journal of hematology Pub Date : 2023-12-01 DOI: 10.14740/jh1188
F. Gragasin, Neel Phaterpekar, Donald Glasgow, H. Sun
{"title":"Successful Ultrasound-Guided Spinal Anesthesia in a Patient With Severe Hemophilia A Undergoing Total Hip Arthroplasty","authors":"F. Gragasin, Neel Phaterpekar, Donald Glasgow, H. Sun","doi":"10.14740/jh1188","DOIUrl":"https://doi.org/10.14740/jh1188","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"19 12","pages":""},"PeriodicalIF":1.2,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139192047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Refractory Pure Red Blood Cell Aplasia Secondary to Major ABO-Incompatible Allogeneic Stem Cell Transplantation Successfully Treated With Daratumumab 达拉单抗成功治疗主要ABO不相容异基因干细胞移植继发的难治性纯红细胞增生症
IF 1.2
Journal of hematology Pub Date : 2023-12-01 DOI: 10.14740/jh1195
Clinton Wu, Pete Manchen, Ariela Edelman, Muhammad Husnain, Emmanuel Katsanis, Deborah Fuchs, Laura Stephens, S. Khurana
{"title":"Refractory Pure Red Blood Cell Aplasia Secondary to Major ABO-Incompatible Allogeneic Stem Cell Transplantation Successfully Treated With Daratumumab","authors":"Clinton Wu, Pete Manchen, Ariela Edelman, Muhammad Husnain, Emmanuel Katsanis, Deborah Fuchs, Laura Stephens, S. Khurana","doi":"10.14740/jh1195","DOIUrl":"https://doi.org/10.14740/jh1195","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"83 2","pages":""},"PeriodicalIF":1.2,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139192724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Peripheral T-Cell Lymphoma in a Patient Previously Diagnosed With Sarcoidosis 一名曾被诊断为肉样瘤病的患者患上外周 T 细胞淋巴瘤
IF 1.2
Journal of hematology Pub Date : 2023-12-01 DOI: 10.14740/jh1173
Sanjay V. Menghani, Jessica P. Diaz-Hanson, Alex Heimbigner, Chelby Wakefield, Deborah Fuchs, Candace Y Reveles, Catherine Spier, Akshay Amaraneni, Abhijeet Kumar
{"title":"Peripheral T-Cell Lymphoma in a Patient Previously Diagnosed With Sarcoidosis","authors":"Sanjay V. Menghani, Jessica P. Diaz-Hanson, Alex Heimbigner, Chelby Wakefield, Deborah Fuchs, Candace Y Reveles, Catherine Spier, Akshay Amaraneni, Abhijeet Kumar","doi":"10.14740/jh1173","DOIUrl":"https://doi.org/10.14740/jh1173","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"63 1-2","pages":""},"PeriodicalIF":1.2,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139194226","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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