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Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

Intensive Care Medicine volume 34, pages 1177–1187 (2008)Cite this article

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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Acknowledgements

The authors thank M.T. Daniel for her contribution to iconography preparation and A. Wolfe for her help with the manuscript.

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  1. Medical ICU and Department of Clinical Immunology, AP–HP, University Paris-7 Diderot, UFR de Médecine, Hôpital Saint-Louis, 1 avenue Claude Vellefaux, 75010, Paris, France

    Caroline Créput, Lionel Galicier, Sophie Buyse & Elie Azoulay

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Créput, C., Galicier, L., Buyse, S. et al. Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med 34, 1177–1187 (2008). https://doi.org/10.1007/s00134-008-1111-y

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  • DOI: https://doi.org/10.1007/s00134-008-1111-y

Keywords

  • Hemophagocytosis
  • Histiocytosis
  • Langerhans cells
  • Th1 Cytokines activation
  • Cytopenia
  • Autoimmune disease