Hematology Reports最新文献

{"title":"Avascular Necrosis of the Femoral Head in Patients with Antiphospholipid Syndrome: A Case Series.","authors":"Paschalis Evangelidis, Eleni Gavriilaki, Nikolaos Kotsiou, Zacharo Ntova, Panagiotis Kalmoukos, Theodosia Papadopoulou, Sofia Chissan, Sofia Vakalopoulou","doi":"10.3390/hematolrep17020015","DOIUrl":"10.3390/hematolrep17020015","url":null,"abstract":"<p><p><b>Background/Objectives</b>: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis or obstetric complications and the laboratory detection of antiphospholipid antibodies. Although vascular thrombosis is the main manifestation of the disease, other rarer complications have also been described. Avascular necrosis (AN) is considered a rare manifestation of APS. The aim of our case series is to study patients with APS and AN. <b>Methods</b>: A retrospective study was performed on 80 patients diagnosed with APS. <b>Results</b>: AN was observed in 3 patients out of 80 diagnosed with APS. AN of the femoral head was observed in all cases. Case (1): A 54-year-old woman presented due to multiple ischemic infarctions in the brain, as detected in magnetic resonance imaging of the brain, Raynaud's phenomenon, and AN of the femoral head. In laboratory testing, a prolongation of activated partial thromboplastin time was recorded. A heterozygous mutation was also found in the gene MTHFR C677T, and the patients was positive for lupus anticoagulant (LA). The patient was given clopidogrel and acenocoumarol. Case (2): A 52-year-old man was diagnosed with APS, based on the clinical presentation (stroke) and positivity for LA and anti-β2GPI (anti-β2 glycoprotein I antibody). In his medical history, episodes of vertigo and an episode of AN of the femoral head 2 years ago were described. Case (3): A woman aged 43 years presented due to AN of the femoral head. Due to suspected APS, immunological testing was performed, and positivity for LA and IgM anticardiolipin antibodies was detected. She was treated with acenocoumarol. <b>Conclusions</b>: AN is a rare clinical manifestation of APS, which may precede the diagnosis of APS for many years.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932201/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691891","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}

{"title":"Pure White Cell Aplasia, an Exceedingly Rare Complication of a Thymoma, and Immune Reconstitution Following Bone Marrow Recovery.","authors":"Giby V George, J C Uy, John L Mariano, Marisa Jacob-Leonce, Chauncey R Syposs","doi":"10.3390/hematolrep17020014","DOIUrl":"10.3390/hematolrep17020014","url":null,"abstract":"<p><strong>Background: </strong>Thymoma-associated pure white cell aplasia (PWCA), characterized by agranulocytosis with absent myeloid precursors in the bone marrow in the setting of preserved erythropoiesis and megakaryopoiesis, is exceedingly rare, with only a few cases reported in the literature. We present a case of type-B2-thymoma-associated PWCA and immune reconstitution following marrow recovery.</p><p><strong>Case presentation: </strong>A 75-year-old woman was incidentally found to have a concomitant mediastinal mass and peripheral leukopenia with absent granulocytes and monocytes. Bone marrow assessment was notable for a hypocellular marrow (<10%) with absent granulopoiesis and monopoiesis. Chest CT demonstrated a large lobulated anterior mediastinal mass, for which the patient underwent a video-assisted thoracoscopic thymectomy. Pathological evaluation of the mediastinal mass specimen revealed a type B2 thymoma. A tentative diagnosis of thymoma-associated PWCA was made, and the patient was started on cyclosporine/granulocyte-colony stimulating factor (G-CSF)/filgrastim therapy. Despite promising marrow recovery, she developed several comorbidities and had a leukemoid reaction, provoking concern for immune reconstitution following prolonged neutropenia and subsequent treatment. She passed away on post-operative day 15, and the results of a post-mortem bone marrow examination were consistent with granulocytic hyperplasia.</p><p><strong>Conclusions: </strong>This case of thymoma-associated PWCA heightens awareness regarding this entity, providing a note of caution regarding the possibility of immune reconstitution following treatment and marrow recovery.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691931","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}

{"title":"Hairy Cell Leukemia: A Differential Diagnosis of Hepatitis B-Associated Aplastic Anemia and Syphilis.","authors":"I Kindekov, E Beleva, M Kadish, I Ionchev, N Semerdzhieva","doi":"10.3390/hematolrep17020013","DOIUrl":"10.3390/hematolrep17020013","url":null,"abstract":"<p><p>Aplastic anemia occurs with an incidence of 2-5: 1 million people worldwide. However, the frequency of newly diagnosed cases of bone marrow aplasia is greater, and some of these patients present to emergency departments initially. <b>Description of Case:</b> We present the case of a middle-aged man with pancytopenia. In this case, aplastic anemia associated with hepatitis B and syphilis was only the initial diagnosis. An indolent hematologic malignancy-hairy cell leukemia-was diagnosed as the real cause of the bone marrow failure in a clinic of hematology. <b>Conclusions:</b> This clinical case allows us to make a conclusion, albeit not definitively, about the contribution of hepatitis B and syphilis to the clinical manifestation of hairy cell leukemia. A detailed and consistent diagnostic plan is also required in patients presenting with pancytopenia. Failure to diagnose a hepatitis B infection in a patient with malignant hematologic disease would lead to fatal therapeutic errors.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691928","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}

{"title":"Acute Intracerebral Hemorrhage Associated with Extensive Venous Thrombosis Due to Spontaneous Heparin-Induced Thrombocytopenia After Total Knee Replacement: A Case Report.","authors":"Mehdi Kashani, Meghan Brown, Juan Pablo Domecq Graces","doi":"10.3390/hematolrep17020012","DOIUrl":"10.3390/hematolrep17020012","url":null,"abstract":"<p><strong>Introduction: </strong>Heparin-induced thrombocytopenia (HIT) is an autoimmune life-threatening prothrombotic syndrome associated with low platelet count after heparin exposure. Spontaneous heparin-induced thrombocytopenia (S-HIT) is an even less frequent variant of HIT, with only a handful of reports available in the literature, where unexplained thrombocytopenia and/or thrombosis without recent heparin exposure occurs in the setting of positive anti-PF4 antibodies.</p><p><strong>Case presentation: </strong>We report a case of S-HIT associated with pulmonary artery embolism, left internal jugular vein, and cerebral vein sinus thrombosis complicated with ipsilateral acute intracerebral hemorrhage.</p><p><strong>Discussion: </strong>It is important to highlight that in patients with otherwise unexplained thrombocytopenia and prior exposure to an inflammatory process, S-HIT should be on the differential.</p><p><strong>Conclusions: </strong>Recognition and avoidance of heparin exposure is the most important aspect of S-HIT, as the management is otherwise similar to HIT.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691884","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}

{"title":"Feasibility of Intensive Chemotherapy in Hereditary Spherocytosis.","authors":"Carrai Valentina, Giubbilei Cristina, Ciceri Manuel, D'Angelo Simona, Nassi Luca, Sordi Benedetta, Vannucchi Alessandro Maria, Puccini Benedetta","doi":"10.3390/hematolrep17020011","DOIUrl":"10.3390/hematolrep17020011","url":null,"abstract":"<p><strong>Background: </strong>This study presents a young man with hereditary spherocytosis (HS) who underwent intensive chemotherapy for newly diagnosed diffuse large B-cell lymphoma (DLBCL) and achieved complete remission. This case challenges the idea of HS as a barrier to standard DLBCL treatment.</p><p><strong>Discussion: </strong>By meticulously monitoring blood counts and providing timely transfusions, the team successfully mitigated potential complications associated with chemotherapy-induced stress on red blood cells.</p><p><strong>Conclusions: </strong>This experience underscores the importance of a multidisciplinary approach and tailored treatment plans for patients with co-existing conditions, suggesting that HS should not automatically disqualify them from potentially curative therapies for aggressive lymphomas.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691921","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}

{"title":"Factor X Concentrate Treatment Schedule and Dosing in Acquired FX Deficiency.","authors":"Andrew Ross, Rebecca J Shaw, Louise Garth, Cathy Farrelly","doi":"10.3390/hematolrep17020010","DOIUrl":"10.3390/hematolrep17020010","url":null,"abstract":"<p><strong>Background: </strong>Acquired factor X (FX) deficiency is a rare condition that can cause life threatening bleeding. Here we outline a successful management strategy for gastrointestinal bleeding (GI) using human FX concentrate.</p><p><strong>Case description: </strong>A 61-year-old male presented with upper GI bleeding and a prolonged prothrombin time. Investigations demonstrated an acquired FX deficiency (determined to be secondary to AL amyloidosis).</p><p><strong>Results: </strong>Treatment with FX concentrate to maintain trough FX levels >20% resulted in successful cessation of bleeding symptoms, and levels >50% facilitated urgent invasive procedures.</p><p><strong>Conclusions: </strong>This case report adds valuable insight into the management of this rare condition, and how best to utilize FX concentrates in acquired FX deficiency.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 2","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691917","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}

{"title":"Awareness and Attitude of the General Population Towards Inherited Hemoglobinopathies in the Premarital Screening Program in the Northern Region of Saudi Arabia.","authors":"Mariah N Hafiz, Nida Suhail, Zakariya M S Mohammed, Husham O Elzein, Hibah A Almasmoum, Awad E Abass, Mohammed M Jawad, Saoussen Trabelsi","doi":"10.3390/hematolrep17010009","DOIUrl":"10.3390/hematolrep17010009","url":null,"abstract":"<p><p><b>Background:</b> Premarital screening (PMS) is a nationwide program that helps high-risk individuals make decisions to avoid genetic and sexually transmitted diseases from spreading to their spouse or future offspring. This study examined the knowledge and attitudes towards inherited hemoglobinopathies in PMS among the people of Northern Border Region in Saudi Arabia and their relationship to various sociodemographic factors. <b>Methods:</b> A cross-sectional study was undertaken in the Northern region of Saudi Arabia from January to March 2024. Data were gathered via questionnaire from 478 Saudi participants aged 18 years and older. The chi-square test was employed to determine the association between categorical variables. <b>Results:</b> All participants in the study were familiar with the PMS program. A significant portion of participants, 79.3%, acknowledged that consanguinity can increase the risk of hereditary blood disorders, while 69.9% believed that if both parents are carriers of the same genetic blood disease, their child may inherit it. Higher education, female gender, and age group (30-40) were found to be the main predictors of knowledge regarding PMS. Most of the participants (98.5%) had a positive attitude regarding the necessity of PMS as a prerequisite for marriage completion. About 82.8% indicated they would not continue with the marriage if the PMS results were incompatible. <b>Conclusions:</b> The study indicates a growing awareness and positive attitude towards premarital screening among the general population, with an increasing number of individuals opting for it. The findings suggest that PMS programs contribute to informed decision making, as evidenced by the rise in participants choosing to forgo marriage due to partner incompatibility. The study recommends the enhancement of health education campaigns by considering demographic factors such as age, education, and marital status. Additionally, it advocates for expanding the scope of PMS to include a wider range of health and genetic disorders to improve its overall efficacy.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11855037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143491755","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}

{"title":"Solid Tumors, Liquid Challenges: The Impact of Coagulation Disorders.","authors":"Nidha Shapoo, Noella Boma, Shobhana Chaudhari, Vladimir Gotlieb","doi":"10.3390/hematolrep17010008","DOIUrl":"10.3390/hematolrep17010008","url":null,"abstract":"<p><p>Coagulation disorders are increasingly recognized as significant complications in patients with solid tumors, affecting morbidity and mortality outcomes. Solid tumors can provoke a hypercoagulable state through the release of pro-coagulant factors, endothelial activation, and inflammation, leading to a heightened risk of coagulation disorders. These coagulation disorders may manifest as venous thromboembolism, arterial thromboembolism, thrombotic microangiopathy, or disseminated intravascular coagulation. These disorders can complicate surgical interventions and impact treatments, including chemotherapy and immunotherapy efficacy, leading to poor outcomes. Understanding the implications of coagulation disorders in solid tumors is essential for optimizing patient management, including identifying high-risk patients, implementing prophylactic measures, elucidating biomarkers for clinical outcomes, and exploring novel therapeutic agents. This review aims to provide insights into the current knowledge surrounding coagulation disorders in solid tumors and their clinical implications.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11854944/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143491763","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}

{"title":"Bosutinib-Induced Pleural Effusion-Class Effect and Cross-Intolerance to All Tyrosine Kinase Inhibitors.","authors":"Nikhil Vojjala, Hizqueel A Sami, Nikhil Kumar Kotla, Supriya Peshin, Kanika Goyal, Soumya Kondaveety, Rishab Rajendra Prabhu, Geetha Krishnamoorthy","doi":"10.3390/hematolrep17010007","DOIUrl":"10.3390/hematolrep17010007","url":null,"abstract":"<p><p><b>Introduction:</b> Tyrosine kinase inhibitors (TKIs) serve as the backbone in the management of chronic myelogenous leukemia and Philadelphia-positive Acute lymphoblastic Leukemia (Ph+ve ALL). With the growing use of TKIs, there has been an increase in adverse events related to these agents. Hereby, we present elderly women with Ph+ve ALL who developed recurrent pleural effusion, which was managed by switching the TKI and highlighting pleural effusion due to a third-generation TKI Bosutinib, adding to the minimal available literature. <b>Case Description:</b> Our patient is a 79-year-old female with Ph+ve ALL diagnosed in 2015 and started on treatment. She is also on TKI maintenance initially with Imatinib later shifted to second-generation TKIs. She started developing worsening dyspnea related to pulmonary toxicity related to TKI in the form of pleural effusion. Pleural effusion was initially managed with diuretics, later requiring thoracocentesis. Because of persistent pleural effusion, she was changed to multiple TKIs and finally started on Bosutinib. She even developed progressive pleural effusion while on Bosutinib which is managed by thoracocentesis. <b>Conclusions:</b> Through this case report, we would like to highlight refractory recurrent pleural effusion caused by bosutinib adding to the minimal available literature. In addition, we highlight the various treatment options in patients having cross-intolerance to various TKIs, especially pulmonary toxicity, and ponatinib might be a suitable option in such cases.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11855369/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143491759","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}

{"title":"Risk Factors for Impaired Glucose Metabolism in Transfusion-Dependent Patients with β-Thalassemia: A Single-Center Retrospective Observational Study.","authors":"Theodora Maria Venou, Filippos Kyriakidis, Fani Barmpageorgopoulou, Stamatia Theodoridou, Athanasios Vyzantiadis, Philippos Klonizakis, Eleni Gavriilaki, Efthymia Vlachaki","doi":"10.3390/hematolrep17010006","DOIUrl":"10.3390/hematolrep17010006","url":null,"abstract":"<p><strong>Background/objectives: </strong>B-thalassemia is a genetic disorder that leads to reduced or absent β-globin chains, often resulting in endocrine abnormalities due to iron overload, chronic anemia, and hypoxia. This study investigates the prevalence and risk factors for glucose metabolism disturbances in transfusion-dependent β-thalassemia (TDT) patients, focusing on pancreatic iron overload and its association with other iron biomarkers.</p><p><strong>Methods: </strong>We studied two groups of TDT patients (2018-2022) at Hippokration General Hospital: Group 1 (no glucose metabolism impairment, n = 46) and Group 2 (with impaired glucose tolerance or diabetes mellitus, n = 18). Patients were assessed for factors contributing to glucose disturbances, and laboratory data were analyzed. Type 2 diabetes was diagnosed per American Diabetes Association criteria, and impaired glucose tolerance was defined by OGTT results. A multivariate logistic regression identified potential independent risk factors. In a subset of patients on iron chelation therapy, we examined the relationship between pancreatic, liver, and heart iron overload (T2* MRI) and glucose/ferritin levels.</p><p><strong>Results: </strong>Age and elevated serum GGT levels were significantly associated with impaired glucose metabolism (<i>p</i> = 0.02). Beta-blocker use was correlated with glucose disturbances (<i>p</i> = 0.02), but multivariate analysis revealed no significant independent risk factors. A significant relationship was found between pancreatic and heart iron overload (r = 0.45, <i>p</i> = 0.04).</p><p><strong>Conclusions: </strong>Elevated GGT levels suggest that oxidative stress and liver dysfunction play a key role in glucose metabolism disturbances. Pancreatic MRI T2* may help predict heart iron overload. Further research is needed to identify reliable biomarkers for glucose regulation in TDT.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"17 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11855877/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143491761","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}