De Schuyter Kelly, Lodewyck Tom
a Internal Medicine , University of Ghent , Ghent , Belgium.
b Department of Haematology , AZ St. Jan Brugge , Bruges , Belgium.
Acta Clin Belg. 2017 Dec;72(6):465-468. doi: 10.1080/17843286.2017.1324599. Epub 2017 May 19.
We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment.
A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis.
The patient was treated with Etoposide, Cyclosporine A, and systemic steroids based on the HLH-2004 protocol. Unfortunately the patient responded very poorly to the treatment with prolonged pancytopenia, leading to central hemorrhage and eventually death.
Diagnosis of hemopagocytic lymphohistiocytosis is difficult and late because of aspecific signs and low incidence. The HLH-criteria can be of use to exclude or confirm diagnosis in unclear cases. Early diagnosis and treatment is crucial given high morbidity and mortality. Therapy depends on type and etiology of the HLH-syndrome. Besides treating the possible triggering factor, Etoposide, steroids, and Cyclosporine A are the mainstays of treatment, sometimes followed by allogenic hematopoietic stem cell transplantation. More research in adults is needed.
我们报告一例噬血细胞性淋巴组织细胞增生症（HLH）病例，以深入了解如何进行诊断及开始恰当治疗。
一名45岁男性因高热和全血细胞减少入住急诊病房。重复骨髓穿刺显示噬血细胞现象。通过广泛检查排除其他感染性和恶性疾病后，最终确诊为噬血细胞性淋巴组织细胞增生症。
根据HLH - 2004方案，该患者接受了依托泊苷、环孢素A和全身性类固醇治疗。不幸的是，患者对治疗反应极差，全血细胞减少持续时间延长，导致中枢性出血，最终死亡。
由于症状不特异且发病率低，噬血细胞性淋巴组织细胞增生症的诊断困难且往往延迟。HLH标准可用于在不明确的病例中排除或确诊。鉴于高发病率和死亡率，早期诊断和治疗至关重要。治疗取决于HLH综合征的类型和病因。除了治疗可能的触发因素外，依托泊苷、类固醇和环孢素A是主要治疗手段，有时随后进行异基因造血干细胞移植。成人患者需要更多研究。
