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早期专科会诊在噬血细胞性淋巴组织细胞增生症诊断和治疗时机中的作用。

The Role of Early Subspeciality Consultation in the Timing of Hemophagocytic Lymphohistiocytosis Diagnosis and Management.

机构信息

From the Division of Rheumatology, John Peter Smith Hospital, Fort Worth, TX.

Division of Rheumatology, Montefiore Medical Center.

出版信息

J Clin Rheumatol. 2022 Mar 1;28(2):e462-e466. doi: 10.1097/RHU.0000000000001759.

Abstract

OBJECTIVE

The aim of this study was to investigate the relation between timing of subspeciality consult and hemophagocytic lymphohistiocytosis (HLH) consideration, immunosuppression initiation, and in-hospital mortality in patients with HLH.

METHODS

We conducted a medical records review study of patients 18 years or older with definite or probable HLH at Montefiore Medical Center between 2006 and 2019. Earlier subspeciality consultation (rheumatology, hematology, and infectious disease) was defined as consultation in less than or equal to 18 hours from time of admission. Demographic, clinical characteristics, and outcomes were compared between patients with early and later subspecialty consultation.

RESULTS

A total of 28 patients were included. The median age was 40 years, and 61% of patients were male. Infection was identified as a cause of HLH in 13 patients (46%). Fifteen patients (54%) were classified as having an earlier subspeciality consultation with a median time (interquartile range) to HLH consideration of 1.0 day (0.3-4.2 days) compared with 7.9 days (3.1-9.9 days) for the later consultation group (p = 0.002). The median time (interquartile range) to immunosuppression initiation was 4.6 days (1.7-7.8 days) versus 10.9 days (5.1-13.4 days) (p = 0.01), respectively. Five patients (33%) had in-hospital deaths in the early consultation group compared with 7 patients (54%) in later consultation group (p = 0.27). Among the subset of patients who survived to discharge, the 90-day readmission rate was higher in the later consultation group (83% vs 30%, p = 0.12).

CONCLUSIONS

In patients with HLH, earlier subspeciality consultation may play a role in earlier HLH consideration and treatment initiation.

摘要

目的

本研究旨在探讨噬血细胞性淋巴组织细胞增生症(HLH)患者的专科会诊时机与 HLH 考虑、免疫抑制治疗开始以及住院死亡率之间的关系。

方法

我们对 2006 年至 2019 年间在蒙特菲奥雷医疗中心(Montefiore Medical Center)就诊的年龄≥18 岁的明确或可能的 HLH 患者进行了病历回顾性研究。早期专科会诊(风湿病学、血液学和传染病学)定义为从入院时间起≤18 小时内的会诊。我们比较了早期和晚期专科会诊患者的人口统计学、临床特征和结局。

结果

共纳入 28 例患者,中位年龄为 40 岁,61%为男性。13 例(46%)患者的 HLH 病因确定为感染。15 例(54%)患者被归类为早期专科会诊,HLH 考虑的中位时间(四分位距)为 1.0 天(0.3-4.2 天),而晚期专科会诊组为 7.9 天(3.1-9.9 天)(p=0.002)。免疫抑制治疗开始的中位时间(四分位距)分别为 4.6 天(1.7-7.8 天)和 10.9 天(5.1-13.4 天)(p=0.01)。早期会诊组有 5 例(33%)患者住院期间死亡,而晚期会诊组有 7 例(54%)(p=0.27)。在存活至出院的患者亚组中,晚期会诊组的 90 天再入院率更高(83% vs 30%,p=0.12)。

结论

在 HLH 患者中,早期专科会诊可能与更早的 HLH 考虑和治疗开始有关。

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