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Article
Hemophagocytic Lymphohistiocytosis: A Series of Five Clinical Cases in Adult Patients at a Single Institution with a Review of the Literature
Gastroenterology
  • David Thomas
  • Nihar Shah, MD, Marshall University
  • Hiren Patel
  • Tapan Pandya
  • Dron Gauchan
  • Michael Maroules
Document Type
Article
Publication Date
1-1-2015
Abstract

Context: Hemophagocytic Lymphohistiocytosis or the “Hemophagocytic Syndrome” is a spectrum of disorders of regulatory immunomodulatory pathways inciting phagocytosis of hematopoietic cells resulting in end-organ damage. The condition appears in both heritable and non-heritable forms from a multitude of possible environmental triggers, most notably infection. The condition often results in a fatal outcome without prompt diagnosis and treatment. Cases in children have been reported much more frequently and classically than in adult patients.

Case Report: In this case series we examined fi ve such cases in adult patients that were found at our institution in a window as small at 2 years with more cases having presented since the time of this writing. In these cases, likely triggers were found ranging from infectious, drug-inducing and even underlying malignancy. The condition can be diagnosed by a set of laboratory and physical criteria (Hemophagocytic Lymphohistiocytosis -2004). Treatment ranges from immunosuppressive agents to chemotherapeutic approaches with variable success.

Conclusion: Clinicians must maintain a higher index of suspicion in cases presenting with ominous symptomatology to ensure a prompt diagnosis and effective treatment of this potentially deadly condition.

Comments

The copy of record is available at http://dx.doi.org/10.4103/1947-2714.166225. Copyright © 2015 North American Journal of Medical Sciences | Published by Wolters Kluwer – Medknow. This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Citation Information
Thomas D, Shah N, Patel H, Pandya T, Gauchan D, Maroules M. Hemophagocytic lymphohistiocytosis: A series of five clinical cases in adult patients at a single institution with a review of the literature. North Am J Med Sci 2015;7:415-20.