Liver changes in reactive haemophagocytic syndrome

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.