Online ISSN: 2515-8260


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Fahim Manzoor, Shareefa Akhter, Nusrat Bashir, Asima Malik, Sheikh Bilal


Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by fever, hepatosplenomegaly, cytopenia, and progressive multiple-organ failure. Secondary HLH is often triggered by autoimmune diseases, malignancy, or infection. Aim: To evaluate cases of hemophagocytosis and to identify the triggering diseases in secondary hemophagocytic lymphohistiocytosis(HLH). Materials and Methods: We evaluated cases of hemophagocytic lymphohistiocytosis over a period of two years in which peripheral smear, bone marrow aspirate, bone marrow biopsy sections and other laboratory diagnostic parameters were studied. Results: The majority of our patients (96.9%) had secondary HLH as opposed to 3.1% with primary HLH. We observed that infection was present in 68.8% cases of secondary HLH and viral infection was the most common aetiology in 20.3% of cases, followed by 12.5% with typhoid as aetiology, 9.4% with hepatitis C, 7.8% with sepsis as etiology and 7.8% with tuberculosis as an etiology. There were 3 deaths reported in secondary HLH, making the mortality rate 4.8% . Conclusion: Haemophagocytic lymphohistiocytosis is are and left threatening disease if left untreated. Mortality is high, even among patients who are treated according to the HLH-2004 protocol. Thus, early recognition and treatment of this disorder is essential to decrease associated morbidity and mortality.

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