What's in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis

Bonnie C. Prokesch, Srikanth Nagalla, Fatemeh Ezzati, Shannan R. Tujios, Arturo Dominguez, Weina Chen, Corey Kershaw, Prapti Patel, Carolina de la Flor, Jeannine Foster, Andrew A. Martin, Maria Teresa de la Morena, Christian A. Wysocki

Research output: Contribution to journalArticle

Abstract

Purpose: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. Methods: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. Results: Of 31 patients included, 67.7% were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48% of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70%. Autoimmune disorders were found in 9.6%. In total, 13 patients survived (44.8%). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. Conclusion: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.

Original languageEnglish (US)
JournalTransfusion and Apheresis Science
DOIs
StateAccepted/In press - Jan 1 2018

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Hemophagocytic Lymphohistiocytosis
Names
Survival
T-Cell Lymphoma
Neoplasms
Therapeutics
Hypoalbuminemia
Electronic Health Records
Interleukin-2 Receptors
Central Nervous System Diseases
L-Lactate Dehydrogenase
Bilirubin
Tertiary Care Centers
Patient Care
Pediatrics
Mortality

Keywords

  • Epstein-Barr virus
  • Hemophagocytic lymphohistiocytosis
  • Lymphoma
  • Soluble IL2 receptor

ASJC Scopus subject areas

  • Hematology

Cite this

@article{6346bf4f7ef148e887b5957f1c005abd,
title = "What's in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis",
abstract = "Purpose: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. Methods: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. Results: Of 31 patients included, 67.7{\%} were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48{\%} of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70{\%}. Autoimmune disorders were found in 9.6{\%}. In total, 13 patients survived (44.8{\%}). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. Conclusion: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.",
keywords = "Epstein-Barr virus, Hemophagocytic lymphohistiocytosis, Lymphoma, Soluble IL2 receptor",
author = "Prokesch, {Bonnie C.} and Srikanth Nagalla and Fatemeh Ezzati and Tujios, {Shannan R.} and Arturo Dominguez and Weina Chen and Corey Kershaw and Prapti Patel and {de la Flor}, Carolina and Jeannine Foster and Martin, {Andrew A.} and {de la Morena}, {Maria Teresa} and Wysocki, {Christian A.}",
year = "2018",
month = "1",
day = "1",
doi = "10.1016/j.transci.2018.10.001",
language = "English (US)",
journal = "Transfusion and Apheresis Science",
issn = "1473-0502",
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}

TY - JOUR

T1 - What's in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis

AU - Prokesch, Bonnie C.

AU - Nagalla, Srikanth

AU - Ezzati, Fatemeh

AU - Tujios, Shannan R.

AU - Dominguez, Arturo

AU - Chen, Weina

AU - Kershaw, Corey

AU - Patel, Prapti

AU - de la Flor, Carolina

AU - Foster, Jeannine

AU - Martin, Andrew A.

AU - de la Morena, Maria Teresa

AU - Wysocki, Christian A.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Purpose: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. Methods: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. Results: Of 31 patients included, 67.7% were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48% of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70%. Autoimmune disorders were found in 9.6%. In total, 13 patients survived (44.8%). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. Conclusion: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.

AB - Purpose: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. Methods: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. Results: Of 31 patients included, 67.7% were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48% of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70%. Autoimmune disorders were found in 9.6%. In total, 13 patients survived (44.8%). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. Conclusion: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.

KW - Epstein-Barr virus

KW - Hemophagocytic lymphohistiocytosis

KW - Lymphoma

KW - Soluble IL2 receptor

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U2 - 10.1016/j.transci.2018.10.001

DO - 10.1016/j.transci.2018.10.001

M3 - Article

C2 - 30327177

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JO - Transfusion and Apheresis Science

JF - Transfusion and Apheresis Science

SN - 1473-0502

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