Chronic granulomatous disease presenting as hemophagocytic lymphohistiocytosis: a case report and review of literature

摘要

Hemophagocytic Lymphohistiocytosis is a potentially fatal disorder characterized by uncontrolled and dysfunctional proliferation of histiocytes and T lymphocytes in the bone marrow, liver and spleen. The disease is also characterized by the presence of hypercytokinemia. The hallmark of this disease is low or absent NK cell activity and decreased cytotoxicity of CD8+ T Lymphocytes. .