噬血细胞淋巴组织细胞增多症:给细胞因子风暴浇上汽油

Kastamonu Medical Journal Pub Date : 2022-06-21 DOI:10.51271/kmj-0036

Ola Abuzaid, Şefika Akyol, Said Can Alcalı, Ekrem Ünal

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引用次数: 0

摘要

持续发热、肝脾肿大、全血细胞减少的患者应考虑有噬血细胞性淋巴组织细胞增多症。高细胞素血症起源于影响细胞毒性T细胞和自然杀伤淋巴细胞活性缺陷的遗传性疾病，是该疾病的潜在病理生理机制。本文综述了近年来在噬血细胞淋巴组织细胞病治疗方面的研究进展。

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Hemophagocytic lymphohistiocytosis: pouring gasoline on the cytokine storm

Hemophagocytic lymphohistiocytosis should be considered in patients with persistent fever, hepatosplenomegaly, pancytopenia. Hypercytokinemia originated from genetic disorder effecting the cellular defects of cytotoxic T and natural killer lymphocyte activity is the underlying pathophysiology of the disorder. In this review we summarized the recent advances in the management of hemophagocytic lymphohistiocytosis.

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Kastamonu Medical Journal

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