Improving Recognition of Secondary Hemophagocytic Lymphohistiocytosis (HLH) through a Ferritin-Based Automated Alert: An Interrupted Time Series Analysis

Le résumé fourni par la source

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by fever, cytopenias, and hyperferritinemia (1). Primary HLH is caused by genetic mutations and typically presents in infancy, while secondary HLH (sHLH) occurs at all ages and is commonly triggered by infections, hematological malignancies, or rheumatic diseases (1). The rarity and nonspecific clinical presentation of sHLH often results in delayed or missed diagnoses (2). Cytopenias are a common feature of sHLH, typically leading clinicians to check hematinics, including ferritin, regardless of whether sHLH is suspected. Although a markedly elevated ferritin concentration in a critically unwell patient strongly suggests sHLH, this association remains under-recognized and represents an opportunity to improve recognition. We aimed to improve sHLH recognition rates in our institution by introducing an automated electronic alert (autoalert), triggered by high ferritin measurements. Values of >6000 µg/L [corresponding to the higher threshold in the HScore, a validated probability tool for HLH (3)] were flagged with a text alert on the sample result. This was supplemented by telephone notifications for levels exceeding 10 000 µg/L. The alert advises clinicians to consider sHLH and links to our institutional guidelines that align with the UK consensus pathway and guidance (4). Ferritin values below this threshold retain a preexisting comment advising clinicians of the typical causes of mild hyperferritinemia (such as liver disease, hemochromatosis, and acute inflammation).

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