Hemophagocytic Lymphohistiocytosis Secondary to Chronic Lymphocytic Leukemia Progression

继发于慢性淋巴细胞白血病进展的噬血细胞性淋巴组织细胞增生症

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作者:Nina Jancar, Filipa Sousa Gonçalves, José Fragoso Duro, Patrício Aguiar, Catarina Jacinto Correia

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an acute, rare systemic hyperinflammatory disorder caused by a dysregulated immune cell function and massive cytokine release, often leading to multiple organ involvement and failure. Fever, hepatosplenomegaly, cytopenia, elevated liver enzymes, hypertriglyceridemia, and hyperferritinemia are the hallmarks of the disease. Its primary (genetic) form is typically observed in pediatric patients and its secondary, acquired form is seen in adult patients with an underlying autoimmune, malignant, or infectious disease. It is not frequently reported in patients with chronic lymphocytic leukemia (CLL) without an infectious or pharmacological trigger. We present a case of a 71-year-old patient with hemophagocytic lymphohistiocytosis due to the progression of chronic lymphocytic leukemia.

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