Hematology Reports最新文献

{"title":"Safe and Effective Administration of Caplacizumab in COVID-19-Associated Thrombotic Thrombocytopenic Purpura.","authors":"Antonella Bruzzese,&nbsp;Ernesto Vigna,&nbsp;Dario Terzi,&nbsp;Sonia Greco,&nbsp;Enrica Antonia Martino,&nbsp;Valeria Vangeli,&nbsp;Francesco Mendicino,&nbsp;Eugenio Lucia,&nbsp;Virginia Olivito,&nbsp;Caterina Labanca,&nbsp;Rosellina Morelli,&nbsp;Antonino Neri,&nbsp;Fortunato Morabito,&nbsp;Francesco Zinno,&nbsp;Antonio Mastroianni,&nbsp;Massimo Gentile","doi":"10.3390/hematolrep15030046","DOIUrl":"https://doi.org/10.3390/hematolrep15030046","url":null,"abstract":"<p><p>Thrombotic thrombocytopenic purpura (TTP) is a potentially life-threatening, rare acute thrombotic microangiopathy (TMA), caused by a severe ADAMTS13 deficiency. As the COVID-19 pandemic rapidly spread around the globe, much data about the pathogenicity of this virus were published. Soon after the detection of the first cases of COVID-19, it was clear that there was a wide range of COVID coagulopathy manifestations, such as deep venous thrombosis, pulmonary thromboembolism, and thrombotic microangiopathies. In the literature, little data have been reported about the association between TTP and COVID-19, and the treatment of COVID-19-associated TTP is still under debate. Here we present the case of a 46-year-old woman who developed a COVID-associated TTP, successfully treated with plasma exchange (PEX), steroids, and caplacizumab.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"448-453"},"PeriodicalIF":0.9,"publicationDate":"2023-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9874664","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 Promyelocytic Leukemia and Brugada Syndrome: A Report on the Safety of Arsenic Trioxide/All-Trans-Retinoic Acid Therapy.","authors":"Giorgio Rosati,&nbsp;Sofia Camerlo,&nbsp;Matteo Dalmazzo,&nbsp;Melissa Padrini,&nbsp;Tiziano Tommaso Busana,&nbsp;Marco De Gobbi,&nbsp;Alessandro Fornari,&nbsp;Alessandro Morotti","doi":"10.3390/hematolrep15030045","DOIUrl":"https://doi.org/10.3390/hematolrep15030045","url":null,"abstract":"<p><p>Acute promyelocytic leukemia (APL) is a rare and aggressive form of acute myeloid leukemia (AML). Instead of cytotoxic chemotherapy, a combination of all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) represents front-line therapy in low-risk patients. However, the therapeutic approach could be challenging in the case of a concomitant diagnosis of Brugada syndrome (BrS), a genetic disease characterized by an increased risk of arrhythmias and sudden cardiac death. Here, we present the case of a BrS patient who has been diagnosed with low-risk APL and treated with ATRA and ATO without observing arrhythmic events. In particular, we highlight the difficulties encountered by clinicians during the diagnostic work-up and the choice of the best treatment for these patients.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"440-447"},"PeriodicalIF":0.9,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9872234","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":"Effects of Sirolimus Treatment on Fetal Hemoglobin Production and Response to SARS-CoV-2 Vaccination: A Case Report Study.","authors":"Maria Rita Gamberini,&nbsp;Cristina Zuccato,&nbsp;Matteo Zurlo,&nbsp;Lucia Carmela Cosenza,&nbsp;Alessia Finotti,&nbsp;Roberto Gambari","doi":"10.3390/hematolrep15030044","DOIUrl":"https://doi.org/10.3390/hematolrep15030044","url":null,"abstract":"<p><p>The β-thalassemias are a group of monogenic hereditary hematological disorders caused by deletions and/or mutations of the β-globin gene, leading to low or absent production of adult hemoglobin (HbA). For β-thalassemia, sirolimus has been under clinical consideration in two trials (NCT03877809 and NCT04247750). A reduced immune response to anti-SARS-CoV-2 vaccination has been reported in organ recipient patients treated with the immunosuppressant sirolimus. Therefore, there was some concern regarding the fact that monotherapy with sirolimus would reduce the antibody response after SARS-CoV-2 vaccination. In the representative clinical case reported in this study, sirolimus treatment induced the expected increase of fetal hemoglobin (HbF) but did not prevent the production of anti-SARS-CoV-2 IgG after vaccination with mRNA-1273 (Moderna). In our opinion, this case report should stimulate further studies on β-thalassemia patients under sirolimus monotherapy in order to confirm the safety (or even the positive effects) of sirolimus with respect to the humoral response to anti-SARS-CoV-2 vaccination. In addition, considering the extensive use of sirolimus for the treatment of other human pathologies (for instance, in organ transplantation, systemic lupus erythematosus, autoimmune cytopenia, and lymphangioleiomyomatosis), this case report study might be of general interest, as large numbers of patients are currently under sirolimus treatment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"432-439"},"PeriodicalIF":0.9,"publicationDate":"2023-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865516","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":"Hearing Loss and Blood Coagulation Disorders: A Review.","authors":"Virginia Corazzi,&nbsp;Andrea Migliorelli,&nbsp;Chiara Bianchini,&nbsp;Stefano Pelucchi,&nbsp;Andrea Ciorba","doi":"10.3390/hematolrep15030043","DOIUrl":"https://doi.org/10.3390/hematolrep15030043","url":null,"abstract":"<p><p>A relationship between microvascular disorders and sensorineural hearing loss (SNHL) has been widely proposed. The vascular hypothesis, theorized for the onset of sudden SNHL (SSNHL), is among the most acknowledged: a localized acute cochlear damage, of ischemic or haemorrhagic nature, could be considered a causative factor of SSNHL. The aim of this review is to assess (i) the effect on hearing in patients affected by blood coagulation disorders (prothrombotic or haemorrhagic) and (ii) the possible etiopathogenetic mechanisms of the related hearing loss. A PRISMA-compliant review was performed. Medline, Embase, and Cinahl databases were searched from inception to 31 January 2023, and a total of 14 studies have been included in the review. The available data suggest that it is possible to consider clotting disorders as a potential condition at risk for sensorineural hearing loss; in particular, coagulation tests and eventually the assessment of genetic and acquired prothrombotic factors should be recommended in patients with SSNHL. Also, an audiological evaluation should be recommended for patients with blood coagulation disorders presenting cochlear symptoms, especially in those suffering from clotting diseases.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"421-431"},"PeriodicalIF":0.9,"publicationDate":"2023-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865515","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":"Fluid Overload-Associated Large B-Cell Lymphoma: A Case Report and Review of Literature.","authors":"Hisham F Bahmad,&nbsp;Aaron S Gomez,&nbsp;Arunima Deb,&nbsp;Fernando Martin Safdie,&nbsp;Vathany Sriganeshan","doi":"10.3390/hematolrep15030042","DOIUrl":"https://doi.org/10.3390/hematolrep15030042","url":null,"abstract":"<p><p>Fluid overload-associated large B-cell lymphoma (FO-LBCL) is a new entity described in the fifth edition of the World Health Organization (WHO) Classification of Hematolymphoid Tumors (WHO-HAEM5). It refers to malignant lymphoma present with symptoms of serous effusions in body cavities (pleural, peritoneal, and/or pericardial) in the absence of an identifiable tumor mass. We present a case of an 82-year-old man with a history of atrial fibrillation and atrial flutter, status post-ablation, essential hypertension (HTN), hyperlipidemia (HLD), and diabetes mellitus (DM) type 2 who was referred to our hospital for shortness of breath due to recurrent pleural effusion. Right video-assisted thoracoscopy with right pleural biopsy was performed. Histopathological examination of the pleural biopsy revealed dense fibrous tissue, chronic inflammation, lymphoid aggregates, and granulation tissue, with no evidence of lymphoma. Cytology of the right pleural fluid revealed large lymphoid cells, which were positive for CD45, CD20, PAX-5, MUM-1, BCL2, BCL6, and MYC protein. They were negative for CD3, CD10, CD138, and HHV-8 by immunohistochemistry (IHC). Epstein-Barr virus (EBV) was negative by in situ hybridization (ISH). Due to the absence of any evidence of lymphoma elsewhere, a diagnosis of fluid overload-associated large B-cell lymphoma (FO-LBCL) was made. We provide a synopsis of the main clinicopathological features of FO-LBCL and the two main differential diagnoses, primary effusion lymphoma (PEL) and diffuse large B-cell lymphoma (DLBCL).</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"411-420"},"PeriodicalIF":0.9,"publicationDate":"2023-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366758/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9872236","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":"Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature.","authors":"Amir F Beirat,&nbsp;Sasmith R Menakuru,&nbsp;Maitri Kalra","doi":"10.3390/hematolrep15030041","DOIUrl":"https://doi.org/10.3390/hematolrep15030041","url":null,"abstract":"<p><p>Hereditary platelet delta (δ)-storage pool deficiency is a rare condition in which there are fewer dense granules in platelets disrupting primary hemostasis. It can cause a mild-moderate bleeding tendency with normal coagulation studies; hence, it is an underdiagnosed diagnostic challenge. The authors present three patients with hereditary platelet delta (δ)-storage pool deficiency who had heavy menstrual bleeding, excessive bleeding following surgery, mucocutaneous bleeding, and a bleeding score greater than or equal to 6. These cases reveal the susceptibility of underdiagnosing platelet disorders and the significance of utilizing a bleeding assessment tool to help guide further workup with transmission electron microscopy to visualize the fewer dense granules in platelets. Although bleeding is typically moderate, it can be severe in certain scenarios, like after mucosal surgeries, and can lead to death, highlighting the importance of the condition's recognition and prophylactic treatment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 3","pages":"405-410"},"PeriodicalIF":0.9,"publicationDate":"2023-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865518","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":"Managing Relevant Clinical Conditions of Hemophilia A/B Patients.","authors":"Massimo Morfini, Jacopo Agnelli Giacchiello, Erminia Baldacci, Christian Carulli, Giancarlo Castaman, Anna Chiara Giuffrida, Giuseppe Malcangi, Angiola Rocino, Sergio Siragusa, Ezio Zanon","doi":"10.3390/hematolrep15020039","DOIUrl":"10.3390/hematolrep15020039","url":null,"abstract":"<p><p>The Medical Directors of nine Italian Hemophilia Centers reviewed and discussed the key issues concerning the replacement therapy of hemophilia patients during a one-day consensus conference held in Rome one year ago. Particular attention was paid to the replacement therapy needed for surgery using continuous infusion (CI) versus bolus injection (BI) of standard and extended half-life Factor VIII (FVIII) concentrates in severe hemophilia A patients. Among the side effects, the risk of development of neutralizing antibodies (inhibitors) and thromboembolic complications was addressed. The specific needs of mild hemophilia A patients were described, as well as the usage of bypassing agents to treat patients with high-responding inhibitors. Young hemophilia A patients may take significant advantages from primary prophylaxis three times or twice weekly, even with standard half-life (SHL) rFVIII concentrates. Patients affected by severe hemophilia B probably have a less severe clinical phenotype than severe hemophilia A patients, and in about 30% of cases may undergo weekly prophylaxis with an rFIX SHL concentrate. The prevalence of missense mutations in 55% of severe hemophilia B patients allows the synthesis of a partially changed FIX molecule that can play some hemostatic role at the level of endothelial cells or the subendothelial matrix. The flow back of infused rFIX from the extravascular to the plasma compartment allows a very long half-life of about 30 h in some hemophilia B patients. Once weekly, prophylaxis can assure a superior quality of life in a large severe or moderate hemophilia B population. According to the Italian registry of surgery, hemophilia B patients undergo joint replacement by arthroplasty less frequently than hemophilia A patients. Finally, the relationships between FVIII/IX genotypes and the pharmacokinetics of clotting factor concentrates have been investigated.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 2","pages":"384-397"},"PeriodicalIF":1.1,"publicationDate":"2023-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10298198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9718084","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 Death or Cardiovascular Events after Acute Coronary Syndrome in Patients with Myeloproliferative Neoplasms.","authors":"Orly Leiva,&nbsp;Andrew Jenkins,&nbsp;Rachel P Rosovsky,&nbsp;Rebecca K Leaf,&nbsp;Katayoon Goodarzi,&nbsp;Gabriela Hobbs","doi":"10.3390/hematolrep15020040","DOIUrl":"https://doi.org/10.3390/hematolrep15020040","url":null,"abstract":"<p><p>Patients with myeloproliferative neoplasms (MPNs) are at increased risk of cardiovascular disease (CVD), including acute coronary syndrome (ACS). However, data on long-term outcomes of patients with MPN who have had ACS and risk factors for all-cause death or CV events post-ACS hospitalization are lacking. We conducted a single-center study of 41 consecutive patients with MPN with ACS hospitalization after MPN diagnosis. After a median follow-up of 80 months after ACS hospitalization, 31 (76%) experienced death or a CV event (myocardial infarction, ischemic stroke, or heart failure hospitalization). After multivariable Cox proportional hazards regression, index ACS within 12 months of MPN diagnosis (HR 3.84, 95% CI 1.44-10.19), WBC ≥ 20 K/µL (HR 9.10, 95% CI 2.71-30.52), <i>JAK2</i> mutation (HR 3.71, 95% CI 1.22-11.22), and prior CVD (HR 2.60, 95% CI 1.12-6.08) were associated with increased death or CV events. Further studies are warranted to improve cardiovascular outcomes in this patient population.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 2","pages":"398-404"},"PeriodicalIF":0.9,"publicationDate":"2023-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10298451/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9710277","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":"Splenic Rupture Secondary to Amyloidosis: A Case Report and Review of the Literature.","authors":"Hisham F Bahmad,&nbsp;Samantha Gogola,&nbsp;Lorena Burton,&nbsp;Ferial Alloush,&nbsp;Mike Cusnir,&nbsp;Michael Schwartz,&nbsp;Lydia Howard,&nbsp;Vathany Sriganeshan","doi":"10.3390/hematolrep15020038","DOIUrl":"https://doi.org/10.3390/hematolrep15020038","url":null,"abstract":"<p><p>Amyloidosis is a term describing the extracellular deposit of fibrils composed of subunits of several different normal serum proteins in various tissues. Amyloid light chain (AL) amyloidosis contains fibrils that are composed of fragments of monoclonal light chains. Many different disorders and conditions can lead to spontaneous splenic rupture, including AL amyloidosis. We present a case of a 64-year-old woman with spontaneous splenic rupture and hemorrhage. A final diagnosis of systemic amyloidosis secondary to plasma cell myeloma was made with infiltrative cardiomyopathy and possible diastolic congestive heart failure exacerbation. We also provide a narrative review of all documented cases of splenic rupture associated with amyloidosis from the year 2000 until January 2023, along with the main clinical findings and management strategies.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 2","pages":"370-383"},"PeriodicalIF":0.9,"publicationDate":"2023-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10298054/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9710279","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":"Role of Therapeutic Anticoagulation in COVID-19: The Current Situation.","authors":"Mandeep Singh Rahi,&nbsp;Jay Parekh,&nbsp;Prachi Pednekar,&nbsp;Mayuri Mudgal,&nbsp;Vishal Jindal,&nbsp;Kulothungan Gunasekaran","doi":"10.3390/hematolrep15020037","DOIUrl":"https://doi.org/10.3390/hematolrep15020037","url":null,"abstract":"<p><p>Thrombotic complications from COVID-19 are now well known and contribute to significant morbidity and mortality. Different variants confer varying risks of thrombotic complications. Heparin has anti-inflammatory and antiviral effects. Due to its non-anticoagulant effects, escalated-dose anticoagulation, especially therapeutic-dose heparin, has been studied for thromboprophylaxis in hospitalized patients with COVID-19. Few randomized, controlled trials have examined the role of therapeutic anticoagulation in moderately to severely ill patients with COVID-19. Most of these patients had elevated D-dimers and low bleeding risks. Some trials used an innovative adaptive multiplatform with Bayesian analysis to answer this critical question promptly. All the trials were open-label and had several limitations. Most trials showed improvements in the meaningful clinical outcomes of organ-support-free days and reductions in thrombotic events, mainly in non-critically-ill COVID-19 patients. However, the mortality benefit needed to be more consistent. A recent meta-analysis confirmed the results. Multiple centers initially adopted intermediate-dose thromboprophylaxis, but the studies failed to show meaningful benefits. Given the new evidence, significant societies have suggested therapeutic anticoagulation in carefully selected patients who are moderately ill and do not require an intensive-care-unit level of care. There are multiple ongoing trials globally to further our understanding of therapeutic-dose thromboprophylaxis in hospitalized patients with COVID-19. In this review, we aim to summarize the current evidence regarding the use of anticoagulation in patients with COVID-19 infection.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"15 2","pages":"358-369"},"PeriodicalIF":0.9,"publicationDate":"2023-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10297876/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9710280","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}