Abstract
The article presents a review of literature sources highlighting the issues of clinical and laboratory diagnosis of hemophagocytic lymphohistiocytosis in children. The authors of various observations have repeatedly emphasized hemophagocytic lymphohistiocytosis continues to be a life-threatening disease despite modern implementations in diagnosis and treatment. At the same time, the prognosis and outcome are largely determined by the timeliness of diagnosis and the right tactic. The review is illustrated by a clinical example of a fatally ended case of an infant, he was diagnosed posthumously based on the results of a molecular genetic tests.
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