• Saad Waheed and Saima Gillani.
• Department of Pediatrics, Ayub Teaching Hospital, Abbottabad, Pakistan.
• J Coll Physicians Surg Pak. 2021 Aug 1; 31 (8): 975-977.

AbstractHemophagocytic Lymphohistiocytosis (HLH) is an uncommon but a potentially fatal disease characterised by high grade fever, hepato-splenomegaly, deranged liver functions, cytopenias and multi-organ involvement. HLH has its own diagnostic criteria, which comprise of various clinical and laboratory features, which were revised in 2004 by HLH Society for prompt identification and recognition. Here, we present a case of 9-year developmentally normal female with all the complaints as mentioned above, who received treatment for almost eight months as a case of pyrexia of unknown origin (PUO) without a definitive diagnosis. The differential diagnosis of HLH was considered in the patient and later, it was corroborated by hemophagocytic activity on bone marrow aspiration examination. The diagnosis of HLH was made after fulfillment of its criteria which was addressed completely and found veracious. After addressing the active complaints of the patient, she was referred to a pediatric oncologist for chemotherapy and further management; and counselled for hematopoietic stem cell transplantation (HSCT). Key Words: Hemophagocytic lymphohistiocytosis, Pyrexia of unknown origin (PUO), Pediatric hematopoietic stem cell transplantation.

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