Journal of hematology最新文献

{"title":"Thymoma With Triple Threat: Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia, and T-Cell Large Granular Lymphocytic Leukemia.","authors":"Tara Seibert,&nbsp;Patrick J Loehrer,&nbsp;Andrew R W O'Brien","doi":"10.14740/jh1061","DOIUrl":"https://doi.org/10.14740/jh1061","url":null,"abstract":"<p><p>Thymomas are a rare neoplasm of the anterior mediastinum and often associated with paraneoplastic syndromes. Though myasthenia gravis is the most common and well-known, the list of reported paraneoplastic syndromes occurring with thymoma is extensive and ever-growing. Paraneoplastic syndromes can involve nearly every organ system, including hematologic abnormalities affecting any or all cell lines. This can present challenges to the clinician in terms of diagnosis, prognostic impact, and management. We present the case of a previously healthy 41-year-old female who was diagnosed with thymoma and three rare hematologic paraneoplastic syndromes: pure red cell aplasia (PRCA), autoimmune hemolytic anemia (AIHA), and T-cell large granular lymphocytic leukemia (T-LGLL). To the best of our knowledge, there have been only four other reported cases of PRCA and AIHA in a single patient with thymoma, all of which were treated with thymectomy. Upfront surgical resection was not possible in the present case and thus the patient was alternatively treated with corticosteroids and octreotide, which proved successful in resolving the anemia. The authors present this case to share these findings of an alternative treatment strategy for thymoma-associated PRCA and AIHA and to highlight the importance of careful monitoring with routine blood work for these complex patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b5/c3/jh-11-223.PMC9822658.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9072691","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":"Synchronous Presentation of Autoimmune Hepatitis and Multiple Myeloma.","authors":"Binoy Yohannan,&nbsp;Allen C Omo-Ogboi,&nbsp;Varaha S Tammisetti,&nbsp;Adan Rios","doi":"10.14740/jh1049","DOIUrl":"https://doi.org/10.14740/jh1049","url":null,"abstract":"<p><p>Autoimmune hepatitis (AIH) is a rare immune-mediated disease predominantly seen in women and triggered by various environmental factors. Rarely, AIH can be triggered by an underlying malignancy. We report a woman in her 60s who presented with markedly abnormal liver biochemical tests. Serology was positive for anti-smooth muscle antibodies and a liver biopsy confirmed AIH. During the hospital course, she developed sepsis and acute renal failure requiring dialysis support. Serum protein electrophoresis (SPEP) showed a monoclonal IgG kappa protein of 1.92 g/dL and a bone marrow biopsy revealed 7% clonal plasma cells. She had lytic lesions on skeletal survey confirming the diagnosis of a coexisting multiple myeloma (MM). Given her markedly abnormal liver chemistries, we decided to treat the AIH first and use the steroids (an important anti-myeloma therapy) as a bridge to the specific treatment of the MM once her clinical condition improved. She was treated with oral prednisone and azathioprine for AIH. One month later, a marked improvement in liver biochemical test results was noted and she was started on oral ixazomib, lenalidomide and dexamethasone. She received palliative radiotherapy to the lumbar spine (L2), left femur, and ischium lesions. This case highlights a rare co-occurrence of AIH and MM, the underlying mechanism of which is unknown.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8f/e2/jh-11-216.PMC9822655.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10524885","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 Myeloid Leukemia Following Myeloproliferative Neoplasms: A Review of What We Know, What We Do Not Know, and Emerging Treatment Strategies.","authors":"Zoe McKinnell,&nbsp;Daniel Karel,&nbsp;Daniel Tuerff,&nbsp;Marwa Sh Abrahim,&nbsp;Samah Nassereddine","doi":"10.14740/jh1042","DOIUrl":"https://doi.org/10.14740/jh1042","url":null,"abstract":"<p><p>Acute myeloid leukemia (AML) arising from myeloproliferative neoplasms (MPNs) represents a small subtype of secondary AML (sAML). This entity is well known to be associated with poor responses to available treatment options and dismal outcomes. To date, there are no standardized treatment options and there has been very little therapeutic advancement in recent years. This is a stark contrast to other subsets of AML for which there have been significant advances in therapeutic approaches, especially for patients with targetable mutations. We aim to focus our review on the incidence, risk factors for leukemogenesis, pathogenesis, molecular landscape, and emerging therapeutic options in post-myeloproliferative neoplasm acute myeloid leukemia (post-MPN AML).</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/85/fb/jh-11-197.PMC9822656.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10578692","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":"Unveiling the Great Therapeutic Potential of MASPs as Hemostatic Agents.","authors":"Ashraf Abdullah Saad","doi":"10.14740/jh1060","DOIUrl":"https://doi.org/10.14740/jh1060","url":null,"abstract":"The lectin complement pathway (LP) is an important effector arm of innate immunity and exemplary pattern recognition artist that draws a fine line between friend and foe (host defense) and between innocuous and noxious (homeostasis). Intriguingly, the proteolytic activity of the LP is attributed to proteolytic enzymes, called mannan-binding lectin (MBL)-associated serine proteases (MASPs). MASPs are central components of the LP that resemble the serine proteases, C1r and C1s, of the classical complement pathway (CP). Recently, the MASPs’ important role in the coagulation cascade was unmasked by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection [1-6]. MASPs (mainly MASP-1) are actually key elements that connect both complement and coagulation systems [7]. So far, research into complement-coagulation interactions focusing merely on MASP inhibition had offered promising targets for novel preventive and therapeutic strategies [8]. Conversely, here I propose that the fibrinolytic activity of MASPs can be explored in the management of bleeding disorders. In particular, traumatic and surgical bleeding are life-threating but potentially avoidable causes of death [9, 10]. Johnston et al showed that postsurgical bleeding is associated with substantial increases in postprocedural length of stay, days spent in critical care, and the risks of infection, vascular events, acute renal failure, and in-hospital mortality [11]. In addition, use of anticoagulants and antiplatelets increases the surgical bleeding risk, creates a need for multiple pharmacologic approaches and poses potential problems in managing surgical patients [1215]. On the other hand, approximately one-third of all trauma patients with bleeding present with a coagulopathy on hospital admission [16, 17]. This subset of patients has a significantly increased incidence of multiple organ failure and death compared to patients with similar injury patterns in the absence of a coagulopathy [18]. Coagulopathy frequently occurs early in the postinjury period and is an independent predictor of mortality. Compared to patients whose initial prothrombin time (PT) and activated partial thromboplastin time (aPTT) are normal, trauma patients have 35% and 326% increased risk of mortality when their initial PT and aPTT are abnormal, respectively [19]. It is important to emphasize that trauma-induced coagulopathy, also called acute traumatic coagulopathy, is distinct from massive transfusion coagulopathy that occurs in the context of loss and dilution coagulopathy [20, 21] or disseminated intravascular coagulation [22]. Hitherto, measures to reduce intraoperative blood loss have been limited to enhancement of coagulation by recombinant activated coagulation factor VII (rFVIIa), desmopressin, fibrinogen, prothrombin complex concentrates, inhibition of fibrinolysis comprising lysine analogues (tranexamic acid and epsilon aminocaproic acid) and a broad-spectrum serine protease inhibitor (aprot","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6a/33/jh-11-240.PMC9822654.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10578687","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":"Real-World Effectiveness of Tixagevimab and Cilgavimab (Evusheld) in Patients With Hematological Malignancies.","authors":"Anthony J Ocon,&nbsp;Kate E Ocon,&nbsp;Jennifer Battaglia,&nbsp;Soon Khai Low,&nbsp;Niraj Neupane,&nbsp;Hassan Saeed,&nbsp;Saad Jamshed,&nbsp;S Shahzad Mustafa","doi":"10.14740/jh1062","DOIUrl":"https://doi.org/10.14740/jh1062","url":null,"abstract":"<p><strong>Background: </strong>Immunocompromised individuals with hematological malignancy have increased risk for poor outcomes and death from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This special population may mount a suboptimal response to vaccination. We assessed the effectiveness of tixagevimab and cilgavimab (Evusheld), a monoclonal antibody combination against SARS-CoV-2, in conjunction with standard preventative measures, at preventing symptomatic incident infection.</p><p><strong>Methods: </strong>Patients aged 18 years and older with hematological malignancy consented to receive Evusheld. Patients were followed longitudinally for development of symptomatic incident SARS-CoV-2 infections. Adverse events were monitored.</p><p><strong>Results: </strong>Two hundred and three patients (94 female) with hematological malignancies and mean age 72 ± 10 years were included. Of the patients, 99.5% had received at least one mRNA vaccination against SARS-CoV-2. Average time of follow-up was 151 ± 50 days. Nineteen patients (9.3%) developed incident symptomatic SARS-CoV-2 infection, with only one (0.5%) requiring hospitalization. During the same follow-up period, local incident rate of infection was 84,123 cases (11.3% of population). Of those, 3,386 cases (4%) of SARS-CoV-2 required hospital admission. The incidence rate ratio was 0.79. No serious adverse events occurred following administration of Evusheld.</p><p><strong>Conclusion: </strong>Patients with hematological malignancy who received Evusheld infrequently developed symptomatic infections or require hospitalization. The high-risk cohort incidence was at least as comparable to the average risk general population. Evusheld appears effective and is well tolerated, and may be administered in conjunction with vaccination and standard prevention measures, at decreasing incident SARS-Co-V2 cases in this high-risk population.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/68/6d/jh-11-210.PMC9822659.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10578686","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":"Bone Involvement as a Primary Rare Manifestation of Waldenstrom Macroglobulinemia: A Case Report and Prevalence in a Nationwide Population-Based Cohort Study.","authors":"Khazra Bhatti,&nbsp;Aqsa Nazir,&nbsp;Simon Ostergaard,&nbsp;Lone Schejbel,&nbsp;Peter Norgaard,&nbsp;Lise M R Gjerdrum,&nbsp;Mahnaz Moghaddas,&nbsp;Torsten H Nielsen,&nbsp;Lars Munksgaard,&nbsp;Lars M Pedersen","doi":"10.14740/jh1073","DOIUrl":"https://doi.org/10.14740/jh1073","url":null,"abstract":"<p><p>Bone involvement is a rare extranodal manifestation in patients with malignant lymphoproliferative diseases and has also been noted as a rare event in patients with Waldenstrom macroglobulinemia (WM). However, the actual prevalence has not been previously reported. We describe an unusual case of a patient with WM who presented with lower back pain and focal bone lesions at initial diagnosis. Magnetic resonance imaging (MRI) revealed multiple vertebral fractures. Positron emission tomography (PET) detected only nodal changes without pathological skeletal-related metabolic activity. Lymph node and bone marrow biopsies combined with an immunoglobulin M (IgM) M component revealed the diagnosis of WM. A next-generation sequencing (NGS) analysis using a targeted lymphoma panel of 59 recurrently mutated genes in lymphoid neoplasms showed mutations in the <i>MYD88</i> and <i>CD79B</i> genes. After treatment with rituximab and bendamustine, the patient achieved a partial remission and pain relief. After 3 years of stable disease, a spontaneous subcapital fracture at the base of the femoral neck and new vertebral compression fractures occurred. Whole-body low-dose computed tomography (WB-LDCT) and bone density (dual energy X-ray absorptiometry (DEXA)) scan revealed marked osteopenia. After insertion of a hip prosthesis, examination of the removed hip showed infiltration of clonal lymphoplasmacytic cells. Our case confirms that one must be aware that bone involvement in patients with WM can occur as a rare manifestation. Interestingly, the <i>MYD88/CD79B</i>-mutated (MCD) genotype in diffuse large B-cell lymphoma is characterized by extranodal involvement and may also be involved in the pathogenesis of skeletal-related disease in the present case. As a follow-up to this unusual case, we have carried out an analysis based on the Danish Lymphoma Registry (LYFO) covering the entire national population in the period 2000 - 2020. The registry study included a cohort of 2,459 patients with WM and lymphoplasmacytic lymphoma. Our data revealed that primary bone involvement at diagnosis occurs in 1.75% of adults with WM. To the best of our knowledge, this is the first report of the prevalence of skeletal-related disease in a large nationwide cohort and defines bone involvement as an exceedingly rare event in WM.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/53/a8/jh-11-233.PMC9822657.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10578688","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":"Dramatic Response After Switching MEK Inhibitors in a Patient With Refractory Mixed Histiocytosis.","authors":"Anais Roeser,&nbsp;Fanelie Jouenne,&nbsp;Laetitia Vercellino,&nbsp;Julien Calvani,&nbsp;Lauriane Goldwirt,&nbsp;Gwenael Lorillon,&nbsp;Abdellatif Tazi","doi":"10.14740/jh1030","DOIUrl":"https://doi.org/10.14740/jh1030","url":null,"abstract":"<p><p>We report the case of a patient with progressive multisystem mixed histiocytosis associating Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) involving the bone marrow, whose lesions harbored the <i>MAP2K1</i> E102-I103del. After initial improvement under the MEK inhibitor trametinib, the treatment was only partially efficient and poorly tolerated. Eventually, although the trough blood level of trametinib at steady state was within expected ranges, the disease progressed to a life-threatening situation, with peritoneal involvement and anasarca. Switching to the MEK inhibitor cobimetinib as a salvage therapy resulted in a dramatic, rapid disease response, and the patient remains disease-free 3 years later with the treatment. The load of the <i>MAP2K1</i> deletion in peripheral blood was correlated with the disease activity and strongly declined with cobimetinib, although it remained detectable at the last follow-up.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f9/28/jh-11-185.PMC9635797.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40716307","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":"Incidence of Venous Thromboembolism in Hospitalized COVID-19 Patients Receiving Thromboprophylaxis.","authors":"Jimmy Huang,&nbsp;Jenny Martinez,&nbsp;Daniel Diaz,&nbsp;William R Wolowich","doi":"10.14740/jh1036","DOIUrl":"https://doi.org/10.14740/jh1036","url":null,"abstract":"<p><strong>Background: </strong>The purpose of this study was to investigate the association between anticoagulant dosing intensity in coronavirus disease 2019 (COVID-19) infected patients and its outcomes on venous thromboembolism (VTE) and all-cause mortality.</p><p><strong>Methods: </strong>This is a retrospective observational study that examined different anticoagulation regimens among COVID-19 patients for prophylaxis of VTE. Primary outcomes of the study were VTE incidence and all-cause mortality for patients receiving prophylaxis-intensity (PPX) and therapeutic-intensity (TX) anticoagulation. Secondary outcomes were incidence of hemorrhagic events and hospital length of stay. Patients were matched (1:1) based on age and Charlson comorbidity score. Sub-group analyses evaluated outcomes within critically ill patients, between specific anticoagulant agents and comorbid conditions.</p><p><strong>Results: </strong>The primary outcome of VTE occurred in six patients within the prophylactic dose group and eight patients in the therapeutic-intensity dose group (risk ratio (RR): 2.02 (95% confidence interval (CI): 0.7 - 5.2); P = 0.2). Bleeding occurred in 15 (11%) patients in the prophylactic group and 27 (19%) patients in the therapeutic group (RR: 0.5 (95% CI: 0.3 - 1.0); P < 0.049). Hospital length of stay was shorter by 4 days in those treated with prophylactic-intensity anticoagulation (P = 0.003). Intensive care unit admission and ventilation were negatively correlated with mortality in a multivariate analysis.</p><p><strong>Conclusions: </strong>Among hospitalized COVID-19 patients, the use of therapeutic-intensity anticoagulation did not show any benefits in reducing the occurrence of VTE. An increase in mortality and in the incidence of hemorrhagic events was statistically significant in the therapeutic-intensity group. Future prospective studies are warranted to evaluate anticoagulation therapy in COVID-19 infected patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3a/77/jh-11-167.PMC9635799.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40716309","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":"Hemoglobin Alpha Chain Variant Zara Associated With Familial Asymptomatic Hypoxemia.","authors":"Anthony Pasquarella,&nbsp;Erin Miller,&nbsp;Edward C C Wong,&nbsp;Masamichi Ito,&nbsp;Marc J Braunstein","doi":"10.14740/jh1028","DOIUrl":"https://doi.org/10.14740/jh1028","url":null,"abstract":"<p><p>Numerous hemoglobin (Hb) gene mutations have been identified, leading to a spectrum of phenotypes ranging from asymptomatic carrier states to complicated hemolytic anemias. We report a rare case of asymptomatic hypoxemia in a father and his teenage daughter both of whom were found to be carriers of Hb gene variant Zara. Workup for alternative cardiovascular causes of hypoxemia was unremarkable. Further sequencing of the alpha globin locus showed both individuals to be heterozygous for the Hb Zara c.274C>A (p.Leu92Ile) variant of unknown significance in the alpha2-globin gene. This is the first documented association of this Hb variant with familial asymptomatic hypoxemia, highlighting the importance of evaluating for hemoglobinopathies in patients with reduced oxygen saturation.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c5/66/jh-11-190.PMC9635801.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40716308","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":"Intravenous Fluid Administration and the Risk of Adverse Outcomes in Sickle Cell Disease Patients Hospitalized for Vaso-Occlusive Crisis.","authors":"Ademola S Ojo,&nbsp;Somtochukwu Ojukwu,&nbsp;Wassihun Asmare,&nbsp;Oluwamayowa Odipe,&nbsp;Daniel Larbi","doi":"10.14740/jh1058","DOIUrl":"https://doi.org/10.14740/jh1058","url":null,"abstract":"<p><p>Vaso-occlusive crisis (VOC) is the leading cause of hospitalization in sickle cell disease (SCD). Intravenous fluid (IVF) administration is the usual practice during VOC episodes to slow the sickling process. In the absence of an evidence-based, clear-cut consensus on the optimal choice, route, and rate of fluid administration, there has been a wide variability in the practice of IVF administration in the treatment of VOC. However, there are growing concerns about the safety of this practice. This systematic review summarized the current evidence on the risk of negative outcomes in SCD patients treated for VOC with IVFs. A database search of Medline/PubMed, EMBASE, Scopus, Web of Science, CINAHL, Wiley Cochrane Library, Clinicaltrials.gov, and conference proceedings of the European Hematology Association (EHA) and American Society of Hematology (ASH) were performed. The results were presented using narrative analysis of quantitative data. Of the 2,821 identified records, a total of three eligible retrospective cohort studies with a total demographic population of 549 SCD patients were included in this review. Normal saline, a frequently used IVF for VOC may be associated with adverse outcomes such as poor pain control and volume overload. Volume overload, new oxygen requirement, acute chest syndrome, and acute kidney injury are potential adverse outcomes of inappropriate IVF use in VOC. There is limited evidence supporting the current practice of IVF use in VOC. Randomized controlled trials are required to fully clarify the place and safety of IVF in the management of VOC.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ca/3d/jh-11-159.PMC9635800.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40716310","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}