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Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

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Applied Physiology in Intensive Care Medicine 2

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Abstract

Objective: This review aims to help critical care clinicians maintain a high level of suspicion regarding the diagnosis of Hemophagocytic Histiolymphocytosis (HLH). It describes the clinical and laboratory features of HLH, outlines its pathophysiology and reviews the most frequent etiologies related to HLH. Prognostic factors and therapeutic options are also reported. Data sources: Review of the literature. Results: The diagnosis of HLH relies on the association of clinical abnormalities and hemophagocytosis in bone marrow, spleen, or lymph node specimens. Liver, pulmonary, renal, cardiac and skin involvement may occur at various degrees possibly leading to multiple organ failure. Three main etiologies can be found, namely infections, lymphoproliferative diseases, or connective tissue diseases. Immune deficiency is often retrieved. Mortality can be as high as 50%. Although clinically mimicking severe sepsis, HLH has a distinct pathophysiology on which specific therapy is based. Early diagnosis and treatment is mandatory to increase the chances of survival. Conclusion: The comprehensive management of severe HLH requires the involvement of a multidisciplinary team in order to determine the best therapeutic strategy and to identify the underlying cause.

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  1. Department of Clinical Immunology, and Hôpital Saint-Louis, Medical ICU, AP-HP, University Paris-7 Diderot, UFR de Médecine, Paris, France

    Caroline Créput, Lionel Galicier, Sophie Buyse & Elie Azoulay

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  2. Lionel Galicier
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Editors and Affiliations

  1. Dept. of Critical Care Medicine, University of Pittsburgh Medical Center, Scaife Hall 606 3550 Terrace Street, Pittsburgh, PA, 15261, USA

    Michael R. Pinsky MD

  2. Dept. Intensive Care Medicine, Hôpital Henri Mondor, 51 av. Maréchal Lattre de Tassigny, 94010, Créteil, CX, France

    Laurent Brochard MD

  3. Dept. Intensive Care Medicine, Hospital de Sant Pau, Avda. S. Antonio M. Claret 167, 08025, Barcelona, Spain

    Jordi Mancebo MD

  4. General Intensive Care Unit, Università Cattolica des Sacro Cuore, Largo A. Gemelli 8, 00168, Rome, Italy

    Massimo Antonelli

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Créput, C., Galicier, L., Buyse, S., Azoulay, E. (2012). Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. In: Pinsky, M.R., Brochard, L., Mancebo, J., Antonelli, M. (eds) Applied Physiology in Intensive Care Medicine 2. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-28233-1_21

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  • DOI: https://doi.org/10.1007/978-3-642-28233-1_21

  • Publisher Name: Springer, Berlin, Heidelberg

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