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Delay in treatment of adult hemophagocytic lymphohistiocytosis is associated with worse in-hospital outcomes

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by uncontrolled activation of the immune system leading to multiorgan failure. Timely initiation of HLH-specific treatment is believed to be essential and lifesaving. Due to the rarity of the condition in adults, there is no data available in the literature to investigate the effects of treatment delay in this age group. We used data from the National Inpatient Sample (NIS) to evaluate the inpatient practices of HLH treatment initiation over 13 years (2007–2019) and their association with clinically relevant inpatient outcomes. Patients were divided into early treatment group (<6 days) and late treatment group (≥ 6 days). We compared outcomes using multivariate logistic regression models adjusting for age, sex, race, and HLH-triggering conditions. There were 1327 and 1382 hospitalizations in the early and late treatment groups, respectively. Hospitalization in the late treatment group had higher rates of in-hospital mortality (OR 2.00 [1.65–2.43]), circulatory shock (OR 1.33 [1.09–1.63]), requiring mechanical ventilation (OR 1.41 [1.18–1.69]), venous thromboembolism (OR 1.70 [1.27–2.26]), infectious complications (OR 2.24 [1.90–2.64]), acute kidney injury (OR 2.27 [1.92–2.68]), and requiring new hemodialysis (OR 1.45 [1.17–1.81]). Additionally, we observed no significant trend in the mean time to treatment over the study period. This study shows the importance of early initiation of HLH treatment and highlights the adverse outcomes of treatment delay.

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Data is publicly available upon request from Healthcare Cost and Utilization Project website.

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Authors and Affiliations

  1. Department of Internal Medicine, Rochester General Hospital, 1425 Portland Avenue, Rochester, NY, 14621, USA

    Ali Abdelhay, Amir Mahmoud, Mariam Mostafa, Tripti Jain & Sheref Elseidy

  2. Department of Internal Medicine, East Tennessee State University, Johnson City, TN, USA

    Suhib Fahmawi

  3. School of Medicine, University of Jordan, Amman, Jordan

    Mouaz Alkasem

  4. Department of Internal Medicine, Henry Ford Hospital, Detroit, MI, USA

    Omar Ammari

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  1. Ali Abdelhay
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Contributions

Contributions: A.A., A.M., M.M., T.J., S.E., and O.A. were involved in the conception and design of the manuscript; S.F. and M.A. collected and revised the required ICD codes; A.A. and A.M. analyzed and interpreted the data. All writers were involved in the writing and approval of the final manuscript.

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Correspondence to Ali Abdelhay.

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Ethics approval

As the NIS data is deidentified, the ethics committee at Rochester General Hospital has determined that no specific ethical approval for this research.

Consent to participate

As the NIS data is deidentified, the ethics committee at Rochester General Hospital has determined that no patient consent is needed for this research. No elements of this work need permission for reproduction.

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The authors declare no competing interests.

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Supplementary information

ESM 1:

Table S1: ICD-10-CM codes used in data gathering. codes are mentioned in detail for ease of replication. Table S2: ICD-9-CM codes used in data gathering. codes are mentioned in detail for ease of replication. Table S3: Sensitivity analyses; excluding patients who received HLH treatment within 48 hours of admission (left), excluding patients with a diagnosed malignancy (middle), excluding patients with underlying connective tissue disease (right).

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Abdelhay, A., Mahmoud, A., Mostafa, M. et al. Delay in treatment of adult hemophagocytic lymphohistiocytosis is associated with worse in-hospital outcomes. Ann Hematol 102, 2989–2996 (2023). https://doi.org/10.1007/s00277-023-05271-w

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  • DOI: https://doi.org/10.1007/s00277-023-05271-w

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