Retrospective Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Crit Care Med. Nov 30, 2018; 7(6): 73-83
Published online Nov 30, 2018. doi: 10.5492/wjccm.v7.i6.73
Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases
Sumit Kapoor, Christopher K Morgan, Muhammad Asim Siddique, Kalpalatha K Guntupalli
Sumit Kapoor, Department of Critical Care Medicine, Montefiore Medical Center, Bronx, NY 10467, United States
Christopher K Morgan, Muhammad Asim Siddique, Kalpalatha K Guntupalli, Department of Pulmonary, Critical Care and Sleep, Baylor College of Medicine, Houston, TX 77030, United States
Author contributions: Kapoor S, Morgan CK designed the study, collected data and participated in writing and revising the manuscript; Siddique MA collected data, did data analysis and reviewed the manuscript; Guntupalli KK reviewed and revised the manuscript.
Institutional review board statement: This study was approved by the Institutional review board of Baylor College of Medicine with IRB No. H-41092.
Informed consent statement: Requirement for written, informed consent was waived off as it is a retrospective chart review based study.
Conflict-of-interest statement: All authors have no conflicts of interest to disclose.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author to: Sumit Kapoor, MD, FCCP, Assistant Professor, Department of Critical Care Medicine, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, United States. drkapoorsumit@gmail.com
Telephone: +1-714-3303466
Received: August 6, 2018
Peer-review started: August 7, 2018
First decision: October 8, 2018
Revised: October 21, 2018
Accepted: November 7, 2018
Article in press: November 7, 2018
Published online: November 30, 2018
Processing time: 130 Days and 13.2 Hours
ARTICLE HIGHLIGHTS
Research background

Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal syndrome increasingly being recognized in intensive care unit (ICU) now. Not many studies have been conducted in an ICU setting to study the complications and outcomes of this patient population.

Research motivation

There is an urgent need for more evidence in literature to help guide Intensivists identify and manage these sick and complicated patients in ICU. This will help to improve their outcomes and decrease complications.

Research objectives

The objective of our research is to study the ICU course, complications and outcomes of adult patients admitted with HLH over the period of 4 years.

Research methods

It is a retrospective observational study of adult patients with HLH admitted to the two academic medical ICUs from 1/1/2013 to 6/30/2017. The diagnosis of HLH was established using HLH-2004 criteria. Data was collected using ICD 9 and 10 codes. Statistical analysis was performed using STATA 15 software.

Research results

Sixteen adult patients were admitted to ICUs over 4 years with HLH with median age of 49 years. Median ICU LOS was 11.5 d and median hospital LOS was 29 d. Septic shock, acute kidney injury (AKI) and acute respiratory failure were the most common ICU complications. Multi system organ failure was the most common cause of death with high mortality of 80% over 90 d. Age (above or below 50 years), Sequential Organ Failure Assessment score on ICU admission, time to diagnose HLH and immune status of patient did not predict mortality.

Research conclusions

Our study showed that HLH in ICU is associated with mortality of 80% over 90 d periods. Most common complications include septic shock, respiratory failure and AKI. Multi system organ failure is the most common cause of death. Clinically significant bleeding and bloodstream infections were also observed in our case series.

Research perspectives

Presentation of HLH in ICU mimics severe sepsis/septic shock. High index of suspicion for HLH is warranted in patients with septic shock and bi/pan cytopenia, not responding to standard treatment. Tests like serum ferritin, fibrinogen, triglycerides, bone marrow/lymph node biopsies help in diagnosis of HLH. Early diagnosis and treatment with chemotherapy is crucial for improved outcomes.