高铁蛋白患者疑似嗜噬性淋巴组织细胞增多症(Hlh)。

Syed Mujtaba Hasnain Nadir, Sheza Arif Toor, Abdul Rahman Albayouk, Moinuddin Mujeeb
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引用次数: 0

摘要

嗜血球性淋巴组织细胞增多症(HLH)诊断困难。易患HLH的情况以类似的方式出现,如败血症和血液学癌症。我们研究了一位66岁的诊断为慢性淋巴细胞白血病的男性,他表现出发热和非特异性症状,包括腹部不适和体重减轻。脓毒症,主要的怀疑被彻底调查和排除。常规的自身免疫病理通过全面的小组检查。这名患者接受了类固醇治疗,据推测,效果有限。在他的血液检查中,最奇怪的是铁蛋白异常高,> 50000。当主治医师根据多年前观察到的类似表现,建议诊断为噬血细胞性淋巴组织细胞增多症时,母临床小组无法解释异常高的铁蛋白。病人开始使用脉冲依托泊苷和地塞米松,然而,不幸的是,他不能恢复。
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Suspecting Haemophagocytic Lymphohistiocytosis (Hlh) In Patients With A High Ferritin.

Heamophagocytic Lymphohistiocytosis (HLH) is problematic to diagnose. The conditions that predispose to HLH present in a similar fashion, such as sepsis and haematological cancers. We look at the case of a 66-year-old man with a diagnosis of CLL, who presented with pyrexia and non-specific symptoms which included abdominal discomfort and weight loss. Sepsis, the principal suspicion was thoroughly investigated and excluded. Routine autoimmune pathologies were exhausted with comprehensive panels. The patient was trialled on steroids, presumptively, with a limited response. What was most peculiar in his blood tests was an unusually high Ferritin of > 50000. The parent clinical team was at a loss to explain the unusually high ferritin when a locum consultant suggested the diagnosis of Haemophagocytic Lymphohistiocytosis based on a similar presentation she had observed many years ago. The patient was started on pulsed Etoposide and Dexamethasone, however, unfortunately, he could not make a recovery.

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