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Year : 2015  |  Volume : 7  |  Issue : 9  |  Page : 415-420

Hemophagocytic lymphohistiocytosis: A series of five clinical cases in adult patients at a single institution with a review of the literature

Department of Medicine, St. Joseph's Regional Medical Center, Paterson, New Jersey, USA

Correspondence Address:
Hiren Patel
Department of Medicine, St. Joseph's Regional Medical Center, 703 Main St., Paterson, New Jersey-07503
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1947-2714.166225

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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 five 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.

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