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      非EB病毒病原体所致感染相关噬血细胞综合征的临床特征及预后 Translated title: Characteristic and prognosis of patients with non-EBV infection-associated hemophagocytic lymphohistiocytosis

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          Abstract

          目的

          探讨非EB病毒病原体所致感染相关噬血细胞综合征(IAHLH)患者的临床特征及预后。

          方法

          收集2015年1月至2021年3月首都医科大学附属北京友谊医院确诊的48例非EB病毒病原体所致IAHLH患者的临床资料,对患者的临床特征、治疗、疗效及预后进行回顾性分析。

          结果

          共纳入48例患者,男28例,女20例,中位年龄34.5(2.0~74.0)岁,导致IAHLH的病原体中,病毒16例(33.3%),细菌17例(35.4%),寄生虫13例(27.1%),真菌2例(4.2%),患者发病至确诊噬血细胞综合征(HLH)的中位时间为40(10~160)d,发病至确诊为IAHLH的中位时间为67(23~270)d。患者起病时临床表现如下:发热48例(100%),脾大34例(70.8%),血细胞减低38例(79.1%),铁蛋白升高45例(93.8%),甘油三酯升高7例(14.6%),纤维蛋白原降低17例(35.4%),NK细胞活性减低26例(59.1%),可溶性CD25升高35例(74.5%)。25例(52.1%)患者起病时伴淋巴结肿大。明确导致HLH的病原体后尽快减停细胞毒药物及激素并应用有效的抗感染治疗,36例(75.0%)患者获得完全缓解,93.3%(14/15)的寄生虫及真菌所致IAHLH患者获得了病情缓解,细菌及病毒相关IAHLH仅有66.7%(22/33)的缓解率。患者5年预期总生存(OS)率为72.3%(95% CI 50.3%~69.8%),多因素分析显示,总胆红素大于2倍正常上限( OR=20.0,95% CI 1.1~378.3, P=0.046)及诱发HLH的病原体感染未控制( OR=19.9,95% CI 2.9~134.5, P=0.002)为预后不良因素。

          结论

          非EB病毒病原体所致IAHLH预后较好,诊断后应尽快减停细胞毒药物及激素,有效控制病原体感染为关键性治疗。

          Translated abstract

          Objective

          To explore the clinical characteristics and outcomes of patients with non-Epstein-Barr virus (EBV) infection-associated hemophagocytic lymphohistiocytosis (IAHLH).

          Methods

          Clinical data of 48 patients diagnosed with non-EBV IAHLH in Beijing Friendship Hospital from January 2015 to March 2021 were collected, and the clinical characteristics, treatment, curative effect and prognosis of the patients were analyzed retrospectively.

          Results

          This study included 48 patients, 28 males and 20 females, with a median(range)age of 34.5(2–74)years. Pathogens that cause IAHLH were as follows: virus(16 cases, 33.3%), bacteria(17 cases, 35.4%), parasitic agents(13 cases, 27.1%), and fungi(2 cases, 4.2%). The median time from onset to diagnosis of hemophagocytic syndrome(HLH)was 40(10–160)days. The median(range)time duration from prodrome to the definite diagnosis of IAHLH was 67(23–270)days. The clinical characteristics were fever(48 cases, 100%), splenomegaly(34 cases, 70.8%), cytopenia(38 cases, 79.1%), elevated ferritin(45 cases, 93.8%), elevated fasting triglyceride levels(7 cases, 14.6%), hypofibrinogenemia(17 cases, 35.4%), decrease natural killer cell activity(26 in 44 cases, 59.1%), and elevated sCD25(35 cases, 74.5%). Twenty-five patients(52.1%)had adenopathy. Once a certain pathogen was identified as the causative factor of hemophagocytic lymphohistiocytosis(HLH), cytotoxic agents and glucocorticoids were withdrawn, and specific pathogen-directed treatment was initiated. After treatment, 36 cases(75.0%)achieved complete response, and 14 of 15 patients(93.3%)with parasitic and fungal HLH got a response; however, the response rate of patient with bacterial and viral HLH was only 66.7%(22 of 33 patients). The estimated 5-year overall survival rate was 72.3%(95% CI 50.3%–69.8%). The adverse prognostic factors were total bilirubin over the upper limit of normal( OR=20.0, 95% CI 1.1–378.3, P=0.046)and pathogenic infection not fully controlled( OR=19.9, 95% CI 2.9–134.5, P=0.002).

          Conclusion

          Non-EBV IAHLH has a good prognosis. When diagnosed, cytotoxic agents and glucocorticoids should be tapered off, and pathogen-targeted therapy should be critically administered to clear the triggering infection.

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          Most cited references21

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          HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

          In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.
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            Adult haemophagocytic syndrome.

            Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage--mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder. Copyright © 2014 Elsevier Ltd. All rights reserved.
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              Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

              Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, whereas the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS; or MAS-HLH). Most information on the diagnosis and treatment of HLH comes from the pediatric literature. Although helpful in some adult cases, this raises several challenges. For example, the HLH-2004 diagnostic criteria developed for children are commonly applied but are not validated for adults. Another challenge in HLH diagnosis is that patients may present with a phenotype indistinguishable from sepsis or multiple organ dysfunction syndrome. Treatment algorithms targeting hyperinflammation are frequently based on pediatric protocols, such as HLH-94 and HLH-2004, which may result in overtreatment and unnecessary toxicity in adults. Therefore, dose reductions, individualized tailoring of treatment duration, and an age-dependent modified diagnostic approach are to be considered. Here, we present expert opinions derived from an interdisciplinary working group on adult HLH, sponsored by the Histiocyte Society, to facilitate knowledge transfer between physicians caring for pediatric and adult patients with HLH, with the aim to improve the outcome for adult patients affected by HLH.
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                Author and article information

                Journal
                Zhonghua Xue Ye Xue Za Zhi
                Zhonghua Xue Ye Xue Za Zhi
                CJH
                Chinese Journal of Hematology
                Editorial office of Chinese Journal of Hematology (No. 288, Nanjing road, Heping district, Tianjin )
                0253-2727
                2707-9740
                February 2022
                : 43
                : 2
                : 128-133
                Affiliations
                [1] 首都医科大学附属北京友谊医院 Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
                Author notes
                通信作者:王昭(Wang Zhao),Email:wangzhao@ccmu.edu
                Article
                cjh-43-02-128
                10.3760/cma.j.issn.0253-2727.2022.02.007
                8980650
                35381673
                64ac2946-6efa-4cdb-8ad3-4a2b9dad0282
                2022年版权归中华医学会所有Copyright © 2022 by Chinese Medical Association

                This work is licensed under a Creative Commons Attribution 3.0 License.

                History
                : 25 June 2021
                Categories
                论著

                噬血细胞综合征,感染,临床特征,预后,hemophagocytic lymphohistiocytosis,infection,clinical characteristics,outcome

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