Tsui W M, Wong K F, Tse C C
Institute of Pathology, Queen Elizabeth Hospital, Hong Kong.
Liver. 1992 Dec;12(6):363-7. doi: 10.1111/j.1600-0676.1992.tb00590.x.
Hepatomegaly and deranged liver functions are common findings in reactive haemophagocytic syndrome (RHS). We report the findings of 12 fatal cases of RHS in which histological materials of the liver are available for study. The underlying diseases of these patients included lymphoma/leukaemia (6 cases), disseminated undifferentiated carcinoma of the ovary (1 case), disseminated nasopharyngeal carcinoma complicated by tuberculosis (1 case), adenovirus pneumonia (1 case), pneumococcal pneumonia (1 case), typhoid fever (1 case), and possible drug intoxication (1 case). Ten patients had involvement of the liver by the underlying disease process which contributed to the marked hepatic derangement. Non-specific reactive hepatitis, sinusoidal dilatation and steatosis resulting from systemic or local effects of the associated diseases and the haemophagocytosis also added to the high incidence of liver abnormalities. A diffuse Kupffer cell hyperplasia with haemophagocytosis is characteristic of the syndrome, as all the cases showed increased numbers of bland-looking histiocytes within the hepatic sinusoids and haemophagocytosis which was moderate to marked in 8 cases and mild in 4. Thus the finding of Kupffer cell hyperplasia with prominent haemophagocytosis in liver biopsy is indicative of an element of RHS and warrants clinical monitoring. Differential diagnoses of haemophagocytosis in liver are also discussed.
肝肿大和肝功能紊乱是反应性噬血细胞综合征（RHS）的常见表现。我们报告了12例RHS死亡病例的研究结果，这些病例均有肝脏组织学材料可供研究。这些患者的基础疾病包括淋巴瘤/白血病（6例）、卵巢弥漫性未分化癌（1例）、播散性鼻咽癌合并肺结核（1例）、腺病毒肺炎（1例）、肺炎球菌肺炎（1例）、伤寒（1例）以及可能的药物中毒（1例）。10例患者的基础疾病累及肝脏，导致明显的肝功能紊乱。相关疾病的全身或局部影响以及噬血细胞现象引起的非特异性反应性肝炎、肝血窦扩张和脂肪变性，也使得肝脏异常的发生率较高。弥漫性库普弗细胞增生伴噬血细胞现象是该综合征的特征，所有病例的肝血窦内均可见数量增多的外观温和的组织细胞，其中8例噬血细胞现象为中度至重度，4例为轻度。因此，肝活检发现库普弗细胞增生伴显著噬血细胞现象提示存在RHS，值得临床监测。本文还讨论了肝脏噬血细胞现象的鉴别诊断。
