Spectrum of Hemophagocytic Lymphohistiocytosis: Experience from a Tertiary Care Centre
Abstract
Background: Hemophagocytic Lymphohistiocytosis (HLH) is a hyperinflammatory syndrome resulting from hypercytokinemia due to highly stimulated but ineffective immune response. Early recognition remains a challenge because its presenting features overlap with many other infective and inflammatory conditions. Prompt identification hence requires a high index of suspicion and detailed analysis of clinical and laboratory findings to arrive at a conclusive diagnosis. At the same time identifying the underlying cause is important as it guides treatment decisions thus reducing morbidity and mortality associated with this disease. Materials and Methods: Consecutive patients meeting the diagnostic criteria for HLH, based on the HLH 2004 protocol of the Histiocyte Society, over a period of one year were included in the study. For these patients clinical features, laboratory parameters and etiological factors were studied. Results: In the course of over one year 16 patients were diagnosed with HLH . Common presenting symptom was fever in all patients. The etiology in our study was mainly secondary to infections namely Dengue-2, Leptospirosis-1, Visceral Leishmaniasis-1, EBV infection=1, Tuberculosis-3 and other causes like Hodgkin lymphoma (NLPHL)-1, SLE-2 and 1 patient with Primary HLH. In 4 cases the etiology was unclear. All patients fulfilled the HLH 2004 criteria. Conclusion: Clinicians should keep a high degree of suspicion in any patient presenting with fever, rapidly developing cytopenias and organomegaly. Also an attempt to identify specific etiology should be made in every patient as early diagnosis and specific treatment helps to reduce the high mortality associated with this disease.References
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