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Myeloma-associated hemophagocytic lymphohistiocytosis – A comprehensive case study and a novel chemotherapy-free approach with anakinra 骨髓瘤相关性嗜血细胞淋巴组织细胞增多症--一项全面的病例研究和使用阿纳金雷的新型免化疗方法
EJHaem Pub Date : 2024-07-04 DOI: 10.1002/jha2.975
Maged Al-Ammari, Danny Hsu, Adam Bryant
{"title":"Myeloma-associated hemophagocytic lymphohistiocytosis – A comprehensive case study and a novel chemotherapy-free approach with anakinra","authors":"Maged Al-Ammari,&nbsp;Danny Hsu,&nbsp;Adam Bryant","doi":"10.1002/jha2.975","DOIUrl":"10.1002/jha2.975","url":null,"abstract":"<p>Hemophagocytic lymphohistiocytosis (HLH) is an immune response syndrome characterized by excessive inflammation and tissue destruction. A limited number of cases involving HLH patients with concomitant multiple myeloma (MM), leading to significant mortality, have been documented, underscoring the importance of timely diagnosis. We present the case of a 78-year-old previously healthy male admitted to our hospital with a newly diagnosed MM. Subsequently, he was diagnosed with HLH and received treatment with anakinra, intravenous immunoglobulin, and dexamethasone. This case report highlights the unique aspect of being the first documented instance of myeloma-associated HLH treated with anakinra.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 5","pages":"1057-1062"},"PeriodicalIF":0.0,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.975","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141678136","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
Cutaneous Langerhans cell histiocytosis and other systemic inflammatory or autoimmune disease manifestations in the setting of clonal hematopoiesis 皮肤朗格汉斯细胞组织细胞增生症和克隆性造血情况下的其他全身炎症或自身免疫性疾病表现
EJHaem Pub Date : 2024-07-04 DOI: 10.1002/jha2.974
Giby V. George, Jane Liesveld, Siba El Hussein, Audrey N. Jajosky
{"title":"Cutaneous Langerhans cell histiocytosis and other systemic inflammatory or autoimmune disease manifestations in the setting of clonal hematopoiesis","authors":"Giby V. George,&nbsp;Jane Liesveld,&nbsp;Siba El Hussein,&nbsp;Audrey N. Jajosky","doi":"10.1002/jha2.974","DOIUrl":"10.1002/jha2.974","url":null,"abstract":"<p>The clinical manifestations and pathophysiology of clonal hematopoiesis (CH)-associated immunological dysfunction are poorly understood. We describe an elderly woman with CH who developed various systemic inflammatory or autoimmune diseases (SIADs), including cutaneous Langerhans cell histiocytosis (LCH) and temporal arteritis. Sequencing of the LCH revealed somatic oncogenic mutations in <i>MAP2K1</i>, <i>IDH2</i>, and <i>SRSF2</i>, with enrichment of the latter two in her peripheral blood at high allele frequencies. These findings raise concern for the future development of a myeloid malignancy. Given the mounting evidence for adult-onset autoinflammatory conditions caused by somatic blood mutations, we suspect CH-mediated immune dysregulation is contributing to her multi-organ involvement by a combination of SIADs.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 5","pages":"1063-1067"},"PeriodicalIF":0.0,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.974","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141678145","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
Weight loss and dysgeusia in relapsed/refractory multiple myeloma patients treated with talquetamab 接受他喹单抗治疗的复发性/难治性多发性骨髓瘤患者体重减轻和消化不良
EJHaem Pub Date : 2024-07-03 DOI: 10.1002/jha2.971
Syed Naqvi, Asis Shrestha, Marah Alzubi, Jawad Alrawabdeh, Sharmilan Thanendrarajan, Maurizio Zangari, Frits van Rhee, Carolina Schinke, Samer Al Hadidi
{"title":"Weight loss and dysgeusia in relapsed/refractory multiple myeloma patients treated with talquetamab","authors":"Syed Naqvi,&nbsp;Asis Shrestha,&nbsp;Marah Alzubi,&nbsp;Jawad Alrawabdeh,&nbsp;Sharmilan Thanendrarajan,&nbsp;Maurizio Zangari,&nbsp;Frits van Rhee,&nbsp;Carolina Schinke,&nbsp;Samer Al Hadidi","doi":"10.1002/jha2.971","DOIUrl":"10.1002/jha2.971","url":null,"abstract":"<p>Talquetamab is an approved therapy for relapsed multiple myeloma. This study examined dysgeusia and weight loss occurrences, alongside investigating symptom reversibility post-treatment cessation. Dysgeusia was prevalent, persisting in 15% of patients. On average, patients lost 6% of their weight during treatment, with weight loss persisting in about half of the patients post-discontinuation. Weight loss and dysgeusia are important adverse events to consider while on talquetamab treatment. Extending dose intervals can potentially prevent such adverse events and should be studied in future prospective clinical trials.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"789-792"},"PeriodicalIF":0.0,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.971","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141682274","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
Acquired glucose-6-phosphate dehydrogenase deficiency after allogeneic stem-cell transplantation 异体干细胞移植后获得性葡萄糖-6-磷酸脱氢酶缺乏症
EJHaem Pub Date : 2024-06-30 DOI: 10.1002/jha2.920
Quentin Vô, Mehdi Khourssaji, Alaa Beshir, Elodie Collinge
{"title":"Acquired glucose-6-phosphate dehydrogenase deficiency after allogeneic stem-cell transplantation","authors":"Quentin Vô,&nbsp;Mehdi Khourssaji,&nbsp;Alaa Beshir,&nbsp;Elodie Collinge","doi":"10.1002/jha2.920","DOIUrl":"https://doi.org/10.1002/jha2.920","url":null,"abstract":"<p>A 29-year-old Caucasian man was treated for acute myeloid leukemia. He underwent allogeneic stem cell transplantation from an unrelated donor. Six months after the transplant, he relapsed, and salvage chemotherapy was administered, including azacitidine and venetoclax. Vitamin C was added to enhance the azacitidine effect. Rasburicase and allopurinol were given to prevent tumor lysis syndrome. The following day, the patient developed a fever, and an influenza infection was diagnosed and treated with oseltamivir.</p><p>On the third day of azacitidine treatment, his blood test showed a hemoglobin level of 6.8 g/dL (range: 13.3–17.6), mean corpuscular volume of 110.6 fL (range: 80.1–99.8), indirect hyperbilirubinemia (indirect bilirubin at 6.35 mg/dL), an undetectable haptoglobin, and a high level of lactate dehydrogenase (1464 IU/L, range: 120–246). These features were suggestive of a hemolytic crisis. A blood smear was performed, showing no schizocytes but revealing hemighost red blood cells (Figure 1), suggesting a glucose-6-phosphate dehydrogenase (G6PD) deficiency. Other causes of hemolysis were excluded, including deficiencies and thrombotic microangiopathy.</p><p>The patient had no personal or family history of a hemolytic crisis. The G6PD activity was low, but the patient had received red blood cell transfusions. The graft donor came from the Middle East. After a few days, the G6PD deficiency was confirmed by the donor medical team, but this information was not transmitted before the allogeneic stem cell transplantation.</p><p>G6PD deficiency, also known as favism, is an X-linked hereditary disease commonly found in African, Asian, Mediterranean, and Middle Eastern regions. It is the most common human enzyme defect, affecting more than 500 million people worldwide. Hemolysis mainly occurs after infection or exposure to oxidant drugs. Rasburicase is known to be a strong trigger of hemolysis in G6PD-deficient patients.</p><p>To our knowledge, this represents the first documented instance of a hemolytic crisis arising from acquired G6PD deficiency subsequent to an allogeneic stem cell transplantation. G6PD deficiency may be transmitted either from a symptomatic donor, regardless of gender, or from an asymptomatic heterozygous female donor following lyonization. The case report highlights the difficulty of establishing a diagnosis of a congenital disease, especially in a low-prevalence country. It underscores the importance of a correct donor evaluation and the necessity of good communication between the patient/donor transplant teams.</p><p>Dr. Vô Quentin: data collection, writing, and editing of the manuscript. Dr. Beshir Alaa: data collection. Dr. Collinge Elodie: critical feedback and manuscript revision. Dr. Khourssaji Mehdi: biological analysis and clinical photography. All authors read and approved the final manuscript.</p><p>The authors declare no conflict of interest.</p><p>The authors received no specific funding for this work.</p><","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"885-886"},"PeriodicalIF":0.0,"publicationDate":"2024-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.920","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994331","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
Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report 静脉注射免疫球蛋白可暂时改善单克隆抗体病引起的获得性冯-威廉综合征:病例报告
EJHaem Pub Date : 2024-06-29 DOI: 10.1002/jha2.969
Kevin G. Zablonski, Aarthi Rajkumar, Lalitha Nayak
{"title":"Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report","authors":"Kevin G. Zablonski,&nbsp;Aarthi Rajkumar,&nbsp;Lalitha Nayak","doi":"10.1002/jha2.969","DOIUrl":"https://doi.org/10.1002/jha2.969","url":null,"abstract":"<p>Acquired von Willebrand syndrome (AVWS) is a bleeding disorder in which an underlying condition induces a quantitative or qualitative deficiency in the von Willebrand factor. This case demonstrates the rare diagnosis of AVWS due to an Immunoglobulin G monoclonal gammopathy in an elderly woman who presented with significant gastrointestinal bleeding. Originally thought to be type 1 von Willebrand disease, this case provides a cautious example to clinicians that without a detailed history or an understanding of the associated laboratory work-up, AVWS may be missed with potentially fatal consequences. Fortunately, AVWS was recognized and treated with intravenous immunoglobulin with a resolution of bleeding.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"833-837"},"PeriodicalIF":0.0,"publicationDate":"2024-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.969","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994333","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
Asteatotic eczema, a cutaneous manifestation of Hodgkin lymphoma in older patients 骨化性湿疹--老年霍奇金淋巴瘤的一种皮肤表现
EJHaem Pub Date : 2024-06-26 DOI: 10.1002/jha2.910
Ariane Le Clainche Compagnie, Charlotte Degoutte, Barbara Papouin, Claire Lamaison, Romain Gounot, Saskia Ingen-Housz-Oro
{"title":"Asteatotic eczema, a cutaneous manifestation of Hodgkin lymphoma in older patients","authors":"Ariane Le Clainche Compagnie,&nbsp;Charlotte Degoutte,&nbsp;Barbara Papouin,&nbsp;Claire Lamaison,&nbsp;Romain Gounot,&nbsp;Saskia Ingen-Housz-Oro","doi":"10.1002/jha2.910","DOIUrl":"https://doi.org/10.1002/jha2.910","url":null,"abstract":"<p>An 82-year-old man was referred for a 13 kg weight loss and an erythroderma evolving for 2 months. Clinical examination revealed an asteatotic eczema (AE) predominant on the trunk (Figure 1A) associated with palmoplantar keratoderma and supracentimetric cervical polyadenopathy. Biologically, the patient presented with 11 g/dL nonregenerative normocytic anemia, neutrophilic polynucleosis of 12.4 G/L, lymphopenia of 0.4 G/L, and hypoalbuminemia of 21 g/L. Imaging (computed tomography [CT] scan and positron emission tomography (PET)-CT) confirmed supradiaphragmatic hypermetabolic polyadenopathy. Skin biopsy showed ichthyosiform dermatosis suggestive of a paraneoplastic process (Figure 1C). Node biopsy confirmed the diagnosis of classic Hodgkin lymphoma (Figure 1D). The patient was treated with brentuximab vedotin alone for the first two perfusions which permitted AE clinical complete remission (CR) (no PET-CT reevaluation was available) and then combined with pembrolizumab for treatment intensification which was effective on both the lymphoma and the dermatosis, and which permitted a CR after four cycles. Treatment was stopped after four doses of brentuximab vedotin and two doses of pembrolizumab because of CR and immunomediated toxicities (nephropathy and pancreatitis). He is still in CR after 10 months of follow-up.</p><p>A 78-year-old man was hospitalized for a diffuse AE associated with palmoplantar keratoderma (Figure 1B). A single firm and painless right inguinal adenopathy measuring 3 cm was found at clinical examination. The CT scan showed primitive and external right iliac adenopathies. Node biopsy was consistent with Epstein-Barr virus-negative classic Hodgkin lymphoma. The patient died suddenly of unknown cause before beginning the treatment.</p><p>AE, first described in 1907 by Bocq, is a particular form of xerosis affecting older patients, which results from a depletion of the secretion of the sebaceous and sweat glands [<span>1</span>]. It may be present in healthy individuals, but certain phenotypic features such as deep, inflammatory cracks with trunk predominance should outweigh possible underlying neoplasia. The association between AE and hemopathies is already described, especially in non-Hodgkin lymphoma [<span>2</span>]. The association with classic Hodgkin lymphoma is rarer. Skin manifestations are present in 17%–53% of patients with classic Hodgkin lymphoma, mainly characterized by non-specific lesions, and can be the disease's gateway [<span>3</span>]. The most frequent skin manifestation is pruritus (20% of cases). Other manifestations include polymorphic skin symptoms, such as prurigo, paraneoplastic pemphigus, erythema multiforme, erythema nodosum, and various kinds of eruptions (bullous, eczematous, and psoriatic). Clinicians should recognize AE by its presentation and be aware that it may reveal Hodgkin lymphoma, especially in older patients.</p><p>The authors declare no conflict of interest.</p><p>No funding has been","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"883-884"},"PeriodicalIF":0.0,"publicationDate":"2024-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.910","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994130","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
Extramedullary chronic phase chronic myeloid leukaemia (CML) involving the central nervous system: A case report 累及中枢神经系统的髓外慢性期慢性髓性白血病(CML):病例报告
EJHaem Pub Date : 2024-06-25 DOI: 10.1002/jha2.967
Akshay Deshpande, Dina Osman, Vidhya Murthy, Hayder Hussein
{"title":"Extramedullary chronic phase chronic myeloid leukaemia (CML) involving the central nervous system: A case report","authors":"Akshay Deshpande,&nbsp;Dina Osman,&nbsp;Vidhya Murthy,&nbsp;Hayder Hussein","doi":"10.1002/jha2.967","DOIUrl":"https://doi.org/10.1002/jha2.967","url":null,"abstract":"<p>Chronic myeloid leukaemia (CML) has been classically described as a disease restricted to the bone marrow with very few reports of extramedullary involvement. CNS involvement with CML has been described in the literature as an aggressive disease in the leukaemic phase either preceding or coexisting with medullary blast crisis or seen in patients with long-term Imatinib therapy. No treatment consensus exists for this patient group and outcomes remain poor. We hereby present a very rare report of CNS involvement with chronic phase CML at diagnosis in a patient who presented with raised intracranial pressure and cranial nerve palsies.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"859-862"},"PeriodicalIF":0.0,"publicationDate":"2024-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.967","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994164","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
Monitoring of molecular responses to tirabrutinib in a cohort of exceptional responders with relapsed/refractory mantle cell lymphoma 监测复发/难治套细胞淋巴瘤特殊应答者队列中对替瑞布替尼的分子反应
EJHaem Pub Date : 2024-06-24 DOI: 10.1002/jha2.966
Abdullah N. M. Alqahtani, Sandrine Jayne, Matthew J. Ahearne, Christopher S. Trethewey, Sai S. Duraisingham, Susann Lehmann, Caroline M. Cowley, Martin J. S. Dyer, Harriet S. Walter
{"title":"Monitoring of molecular responses to tirabrutinib in a cohort of exceptional responders with relapsed/refractory mantle cell lymphoma","authors":"Abdullah N. M. Alqahtani,&nbsp;Sandrine Jayne,&nbsp;Matthew J. Ahearne,&nbsp;Christopher S. Trethewey,&nbsp;Sai S. Duraisingham,&nbsp;Susann Lehmann,&nbsp;Caroline M. Cowley,&nbsp;Martin J. S. Dyer,&nbsp;Harriet S. Walter","doi":"10.1002/jha2.966","DOIUrl":"https://doi.org/10.1002/jha2.966","url":null,"abstract":"<p>To the Editor,</p><p>Inhibitors of Bruton's tyrosine kinase (BTK) in chronic lymphocytic leukemia (CLL) result in durable responses in nearly all patients [<span>1</span>]. In contrast, in more aggressive B-cell malignancies, including diffuse large B cell lymphoma (DLBCL) and mantle cell lymphoma (MCL), responses to single agent BTK inhibitors (BTKi) occur only in subsets of patients and are mostly of brief duration. However, exceptional responses may occur [<span>2-6</span>].</p><p>In relapsed/refractory (R/R) MCL, median progression-free survival (PFS) with single-agent covalent BTKi range from 12 (ibrutinib) to 20 months (acalabrutinib ACE-LY-004 NCT02213926 study) (Table S1) [<span>7-9</span>]. Analysis of responding patients in ACE-LY-004 showed that 8/29 evaluable patients eradicated minimal residual disease (MRD) using the quantitative ClonoSEQ next-generation sequencing assay of cellular peripheral blood DNA [<span>10</span>]. However, molecular determinants of responsiveness to BTKi in MCL, and clinical significance of attaining MRD negativity in this setting remain unknown.</p><p>Like acalabrutinib, tirabrutinib is a highly selective BTKi, binding covalently to BTKC481 via a reactive acyl alkyne group. Although only 16 MCL patients were treated with single-agent tirabrutinib within the POE001 phase 1 trial (NCT01659255), extended follow-up showed an estimated median PFS of 25.8 months at 3 years [<span>11</span>]. Three patients from this cohort, described below, attained complete responses lasting over 72 months, despite adverse prognostic features at diagnosis including <i>TP53</i> mutations, blastoid morphology, and refractoriness to immunochemotherapy. In these exceptional responders [<span>12</span>], we sought to determine common features or a tumoral mutational signature that might predict exceptional responsiveness. Secondly, we determined the depth of response using digital droplet PCR (ddPCR) in both plasma and cellular DNA samples and whether serial assessment of levels of cellular and plasma circulating tumor (ct) DNA samples might presage relapse.</p><p>Clinical and laboratory details of the three cases are given in Table 1. A schema of the treatment timelines is shown in Figure 1. Full materials and methods are found in the Supporting Information. The three patients (201-139, 201-162, and 201-170) were all treated at our center and received either 480 or 600 mg of tirabrutinib once per day. All entered a clinical and radiological remission. One patient (patient 201-162) with primary immunochemotherapy-refractory disease remains in complete remission (CR) &gt; 108 months from trial initiation. However, in 2020, they developed estrogen receptor positive grade 2 invasive ductal breast cancer, necessitating temporary discontinuation of tirabrutinib for 4 months. The breast cancer was treated radically with surgical resection, post operative radiotherapy and tamoxifen. There remains no clinical or radiological evidence of r","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"896-899"},"PeriodicalIF":0.0,"publicationDate":"2024-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.966","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994043","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
Fatal drug reaction to andexanet alfa: a case report 对安赛蜜α的致命药物反应:一份病例报告
EJHaem Pub Date : 2024-06-22 DOI: 10.1002/jha2.959
Richard J. Buka, Mamidipudi T. Krishna, David J. Sutton
{"title":"Fatal drug reaction to andexanet alfa: a case report","authors":"Richard J. Buka,&nbsp;Mamidipudi T. Krishna,&nbsp;David J. Sutton","doi":"10.1002/jha2.959","DOIUrl":"https://doi.org/10.1002/jha2.959","url":null,"abstract":"<p>Andexanet alfa is a recombinant, modified factor Xa (FXa) molecule that is used for the reversal of the anticoagulant effect of oral anti-FXa anticoagulants in patients with major haemorrhage. Here, we present a case of an 85-year-old man taking rivaroxaban for atrial fibrillation, who presented with an acute, upper gastrointestinal bleed. He was stabilised with red cell transfusion and then received a 400 mg bolus of andexanet alfa. Within minutes of this, he developed chest tightness, shortness of breath, ischaemic electrocardiographic changes and then cardiac arrest from which he could not be resuscitated. The onset of symptoms was clearly temporally related to andexanet alfa administration and the differential diagnosis includes anaphylaxis with Kounis syndrome, or myocardial infarction. Although infusion site reactions have been reported and are relatively common, this is to date the first case of a fatal drug reaction andexanet alfa. This knowledge can be factored into physicians’ risk–benefit decisions when treating patients with oral anti-FXa anticoagulant-associated major haemorrhage.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"829-832"},"PeriodicalIF":0.0,"publicationDate":"2024-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.959","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994327","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
Fostamatinib for immune thrombocytopenic purpura in adult patients: A systematic review and meta-analysis 福斯他替尼治疗成人免疫性血小板减少性紫癜:系统回顾与荟萃分析
EJHaem Pub Date : 2024-06-21 DOI: 10.1002/jha2.939
Roger Kou, Lucy Zhao, Daniel Tham, Rachael Principato, Giovanna Schünemann, Aqib Mannan, Mark Crowther
{"title":"Fostamatinib for immune thrombocytopenic purpura in adult patients: A systematic review and meta-analysis","authors":"Roger Kou,&nbsp;Lucy Zhao,&nbsp;Daniel Tham,&nbsp;Rachael Principato,&nbsp;Giovanna Schünemann,&nbsp;Aqib Mannan,&nbsp;Mark Crowther","doi":"10.1002/jha2.939","DOIUrl":"https://doi.org/10.1002/jha2.939","url":null,"abstract":"<p>Immune thrombocytopenic purpura (ITP) is an immune disorder characterized by thrombocytopenia. Fostamatinib is an orally administered spleen tyrosine kinase inhibitor intended to treat refractory ITP. To evaluate the efficacy and safety of fostamatinib as a subsequent-line therapy for ITP in adults. We searched four electronic databases for primary studies of any design. Primary efficacy outcomes included proportions of patients achieving overall (≥30 × 10<sup>9</sup> cells/L), partial (≥50 × 10<sup>9</sup> cells/L), and stable (as defined in original studies) platelet response. Safety outcomes included rescue medication use and other adverse events. We used narrative synthesis and Mantel–Haenszel random effect meta-analysis to summarize results. Our systematic review included 11 studies for analyses (<i>n</i> = 722). Weighted mean proportions of patients achieving overall, partial, and stable responses with fostamatinib treatment were 0.70 [0.62, 0.76], 0.48 [0.36, 0.61], and 0.28 [0.16, 0.44], respectively. Fostamatinib was favored over placebo for partial (relative risk [RR] = 3.04, 95% confidence interval [CI] [1.53, 6.06]) and stable (RR = 6.43, 95% CI [1.58, 26.23]) responses. Patients on fostamatinib required less rescue medication and were more likely to experience hypertension. Fostamatinib is a viable subsequent-line therapy option for refractory ITP. Given the heterogeneous data and large number of small studies, these results should be interpreted cautiously.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 4","pages":"651-660"},"PeriodicalIF":0.0,"publicationDate":"2024-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.939","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141994325","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
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