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Hematologists are often part of a multispecialty team that is asked to evaluate critically ill patients with suspected hemophagocytic lymphohistiocytosis (HLH). In adult patients, HLH is often secondary to infection, malignancy, or underlying autoimmune diseases. However, diagnostic and treatment protocols pertain primarily to pediatric patients, who are likely to have primary HLH. Here, an international expert panel provides recommendations for the diagnosis and management of HLH in adult patients.
Diagnosis is based on pediatric protocols such as HLH-2004 along with clinical judgement and patient history.
Although hyperferritinemia often triggers the workup, this parameter i…