{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T18:38:07Z","timestamp":1771353487011,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T00:00:00Z","timestamp":1623456000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Lab Hematology"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Hemophagocytic lymphohistiocytosis (HLH) is an immune deregulation disorder with varied clinical presentation which clinically overlaps with widespread tropical infections.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a retrospective chart review of children diagnosed with HLH at our center from February\u20102017 to October\u20102020.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Out of the nine diagnosed patients, genetic predisposition was present in three children; two had identified infectious triggers. The mean age of presentation was 30\u00a0months with male predominance. The most common clinical findings were fever, organomegaly, and pancytopenia. The median value of fibrinogen was\u2010156\u00a0mg\/dL, ferritin\u201012\u00a0957\u00a0ng\/mL and for triglycerides\u2010349\u00a0mg\/dL, respectively. In children with identified genetic predisposition, serum ferritin levels were usually more than 10\u00a0000\u00a0ng\/mL. The majority of our patients had evidence of hemophagocytosis on bone marrow examination. In our experience, although nonspecific, very high ferritin and serum triglycerides with low fibrinogen in a patient with bi\u2010cytopenia, pancytopenia was the most suggestive evidence of HLH. Genetic evaluation in our series identified three children, one with primary HLH genetic mutation and two with underlying immune deficiency syndrome. The presence of HLH in the accelerated phase of Chediak\u2010Higashi and AD Hyper IgE syndrome with HLH is extremely rare. Leishmaniasis (in nonendemic area) and Ebstein\u2010Barr virus (EBV) was identified as an infectious trigger in two cases. Most of our cases received treatment as per HLH 2004 protocol. Three children died during the initial diagnosis and treatment. HLH with subcutaneous panniculitis\u2010like T\u2010cell lymphoma recovered well.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>HLH remains a life\u2010threatening disorder associated with a variety of underlying illnesses as highlighted by our case series.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijlh.13626","type":"journal-article","created":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T13:49:51Z","timestamp":1623505791000},"page":"1443-1450","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["A hungry Histiocyte, altered immunity and myriad of problems: Diagnostic challenges for Pediatric HLH"],"prefix":"10.1111","volume":"43","author":[{"given":"Akriti","family":"Gera","sequence":"first","affiliation":[{"name":"Department of Pediatrics VMMC and Safdarjung Hospital New Delhi India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2884-3600","authenticated-orcid":false,"given":"Aroonima","family":"Misra","sequence":"additional","affiliation":[{"name":"Indian Council of Medical Research National Institute of Pathology New Delhi India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1797-447X","authenticated-orcid":false,"given":"Aditi","family":"Tiwari","sequence":"additional","affiliation":[{"name":"Department of Pediatrics VMMC and Safdarjung Hospital New Delhi India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4440-5339","authenticated-orcid":false,"given":"Amitabh","family":"Singh","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Department of Pediatrics VMMC and Safdarjung Hospital New Delhi 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