Esmaili Heidarali, Rahmani Omid, Fouladi Rohollah F
Department of Pathology, Tabriz University of Medical Sciences, Tabriz, Iran.
Turk Patoloji Derg. 2013;29(1):15-8. doi: 10.5146/tjpath.2013.01142.
To investigate the frequency of hemophagocytic syndrome in a series of patients with otherwise unexplained cytopenia.
In this cross-sectional, single-centre study, bone marrow specimens (n=288) were obtained from the patients with unexplained cytopenia. The diagnosis of hemophagocytic syndrome was made according to universally accepted criteria. Characteristics of the patients, as well as the clinical and laboratory findings were reported.
Fifteen cases (5.2%) fulfilled the hemophagocytic syndrome criteria, including 8 males (53.3%) and 7 females (46.7%) with a mean age of 39.7±20.7 (range: 14-72) years at the time of diagnosis. The main clinical and laboratory findings were cytopenia (100%), fever (73.3%), hyperferritinemia (66.7%), elevated erythrocyte sedimentation rate (60%), hypertriglyceridemia (60%), organomegaly (53.3%), elevated liver enzymes (53.3%), lymphadenopathy (26.7%), neurological symptoms (20%), and skin rash (13.3%). Two patients (13.3%) died before a diagnosis was made.
Our findings indicate that the hemophagocytic syndrome is not a rare pathologic condition in patients with otherwise unexplained cytopenia. Without treatment, the mortality rate may be high.
调查一系列原因不明的血细胞减少症患者中噬血细胞综合征的发生率。
在这项横断面单中心研究中，从原因不明的血细胞减少症患者中获取骨髓标本（n = 288）。噬血细胞综合征的诊断依据普遍接受的标准进行。报告了患者的特征以及临床和实验室检查结果。
15例（5.2%）符合噬血细胞综合征标准，其中男性8例（53.3%），女性7例（46.7%），诊断时平均年龄为39.7±20.7岁（范围：14 - 72岁）。主要临床和实验室检查结果包括血细胞减少（100%）、发热（73.3%）、高铁蛋白血症（66.7%）、红细胞沉降率升高（60%）、高甘油三酯血症（60%）、脏器肿大（53.3%）、肝酶升高（53.3%）、淋巴结病（26.7%）、神经症状（20%）和皮疹（13.3%）。2例患者（13.3%）在确诊前死亡。
我们的研究结果表明，噬血细胞综合征在原因不明的血细胞减少症患者中并非罕见的病理状况。未经治疗，死亡率可能较高。
