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Hemophagocytic lymphohistiocytosis in Egyptian children: diagnosis, treatment challenges and outcome.

BACKGROUND: Hemophagocyticlymphohistiocytosis (HLH) is a spectrum of immune activation which could be genetically determined, or secondaryto an underlying illness. Our aim was to present the clinico-genetic aspects ofHLH among Egyptian children and to evaluate the patterns of reactivation and outcomewith illustrations of overlap manifestations.

RESEARCH DESIGNAND METHODS: We retrospectively collected the data of 55patients with HLH, registered at Ain Shams University Children's Hospital,Cairo, Egypt.

RESULTS: Median age at diagnosis was 19 months (range 2-180), 33 patients (60%)fulfilled the diagnostic HLH criteria at presentation. Fourteen (25.45%) patients had secondary HLH, 15 (27.27%) patients had geneticallydocumented familial HLH (11 had variants in UNC13D gene and one in PRF1 gene), 3 had Griscelli and Chediak-Higashi syndrome. Sixteen patients (29.1%) had reactivations, 8(50%) of themhad molecularly confirmed HLH. Wereport the death of 40 patients, the median duration from the diagnosis todeath of 5 months mostly due to disease activity.

CONCLUSIONS: This study confirms that the nonspecific signsand symptoms of HLH arechallenging. Genetic testing, though expensive and sophisticated, is integralfor the diagnosis. The difficulty in finding non-related donors for stem celltransplantation and the early reactivations are the causes of the inferioroutcome.

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