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  1. Copilot 答案
    Signs and symptoms

    HLH as defined by the HLH-04 criteria is a descriptive diagnosis. Its components are non-specific.
    The onset of HLH occurs before the age of one year in approximately 70 percent of cases. Familial … 展开

    Causes

    The vast majority of patients who meet these criteria will not have a genetic cause of their disease, but rather HLH will be triggered by infection, malignancy, rheumatic disease, and/or certain treatments (as i… 展开

    Pathophysiology

    The underlying causes, either inherited or acquired, lead to an unchecked immune response when exposed to triggers. Impaired NK-cell cytotoxicity is the hallmark of HLH. All genetic defects for familial HLH are related to … 展开

    Diagnosis

    The blood count typically shows decreased numbers of blood cells—including a decreased number of circulating red blood cells, white blood cells, and platelets. The bone marrow may show hemophagocytosis. The liver functio… 展开