Pediatric hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening condition with significant diagnostic and therapeutic difficulties. The purpose of this study was to describe the clinical presentation, the diagnostic challenges, and the outcomes of HLH in children assessed at Mukalla hospital.
Data from 20 medical records of HLH patients admitted between January 2010 and May 2022 were retrospectively analyzed.
The median age at presentation was 3.5 ± 5.1 years. M: F ratio was 1:1. The median time for referral to the hospital was 30±64 days. The most common clinical manifestations were fever and pallor in 95% of cases, and splenomegaly (85%). Hepatomegaly, chest, renal, and neurological manifestations were detected in 80%, 45%, 15%, and 20% of cases, respectively. Bone marrow (BM) hemophagocytosis was detected in 60 % of cases. Sixteen patients fulfilled the HLH diagnostic criteria, and eleven patients (55%) received the HLH 2004 protocol. Out of the 20 patients, three (15%) patients are alive. Fourteen patients died, with overall mortality of 82.35%. All mortalities were due to HLH disease with multi-organ failure. Relapse was noticed in five patients either during treatment or after full recovery.
Pediatric HLH is a challenging emergency with a high mortality rate. High clinical suspicion is essential for early detection to facilitate early intervention to improve the prognosis.
Key words: Key words: cytokine, familial, ferritin, hemophagocytosis, hemophagocytic lymphohistiocytosis, pancytopenia.
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