Limitations of HLH-2004 criteria in distinguishing malignancy-associated hemophagocytic lymphohistiocytosis

Arun Gurunathan; Alexander A. Boucher; Melissa Mark; Kristina M. Prus; Maureen M. O'Brien; Erin H. Breese; Benjamin E. Mizukawa; Michael J. Absalon; Adam S. Nelson; Michael B. Jordan; Michael S. Grimley; Robert B. Lorsbach; Seth J. Rotz; Reema Mathanda; Ashish R. Kumar

doi:10.1002/pbc.27400

Limitations of HLH-2004 criteria in distinguishing malignancy-associated hemophagocytic lymphohistiocytosis

Arun Gurunathan, Alexander A. Boucher, Melissa Mark, Kristina M. Prus, Maureen M. O'Brien, Erin H. Breese, Benjamin E. Mizukawa, Michael J. Absalon, Adam S. Nelson, Michael B. Jordan, Michael S. Grimley, Robert B. Lorsbach, Seth J. Rotz, Reema Mathanda, Ashish R. Kumar

Research output: Contribution to journal › Article › peer-review

25 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is characterized by dysregulated immune activation. Primary HLH involves hereditary deficits in cytotoxic lymphocytes while secondary HLH is triggered by extrinsic factors. The HLH-2004 criteria are widely used for clinical diagnosis, yet their specificity for HLH or their ability to differentiate primary from secondary disease is unclear, potentially leading to inappropriate treatment. We describe several cases where fulfillment of HLH-2004 criteria obscured the diagnoses of underlying malignancies and delayed curative management. These issues are remedied without waiting for genetic testing results through an alternative diagnostic approach using flow cytometry–based immunologic assays and a thorough investigation for malignancy.

Original language	English (US)
Article number	e27400
Journal	Pediatric Blood and Cancer
Volume	65
Issue number	12
DOIs	https://doi.org/10.1002/pbc.27400
State	Published - Dec 2018
Externally published	Yes

Bibliographical note

Publisher Copyright:
© 2018 Wiley Periodicals, Inc.

Keywords

diagnostic approach
hemophagocytic lymphohistiocytosis
oncology

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This output contributes to the following UN Sustainable Development Goals (SDGs)

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Gurunathan, A., Boucher, A. A., Mark, M., Prus, K. M., O'Brien, M. M., Breese, E. H., Mizukawa, B. E., Absalon, M. J., Nelson, A. S., Jordan, M. B., Grimley, M. S., Lorsbach, R. B., Rotz, S. J., Mathanda, R., & Kumar, A. R. (2018). Limitations of HLH-2004 criteria in distinguishing malignancy-associated hemophagocytic lymphohistiocytosis. Pediatric Blood and Cancer, 65(12), Article e27400. https://doi.org/10.1002/pbc.27400

Gurunathan, A, Boucher, AA, Mark, M, Prus, KM, O'Brien, MM, Breese, EH, Mizukawa, BE, Absalon, MJ, Nelson, AS, Jordan, MB, Grimley, MS, Lorsbach, RB, Rotz, SJ, Mathanda, R & Kumar, AR 2018, 'Limitations of HLH-2004 criteria in distinguishing malignancy-associated hemophagocytic lymphohistiocytosis', Pediatric Blood and Cancer, vol. 65, no. 12, e27400. https://doi.org/10.1002/pbc.27400

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abstract = "Hemophagocytic lymphohistiocytosis (HLH) is characterized by dysregulated immune activation. Primary HLH involves hereditary deficits in cytotoxic lymphocytes while secondary HLH is triggered by extrinsic factors. The HLH-2004 criteria are widely used for clinical diagnosis, yet their specificity for HLH or their ability to differentiate primary from secondary disease is unclear, potentially leading to inappropriate treatment. We describe several cases where fulfillment of HLH-2004 criteria obscured the diagnoses of underlying malignancies and delayed curative management. These issues are remedied without waiting for genetic testing results through an alternative diagnostic approach using flow cytometry–based immunologic assays and a thorough investigation for malignancy.",

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Limitations of HLH-2004 criteria in distinguishing malignancy-associated hemophagocytic lymphohistiocytosis

Abstract

Bibliographical note

Keywords

UN SDGs

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