Liver changes in reactive haemophagocytic syndrome

Area of Science:

• Hepatology
• Hematology
• Pathology

Background:

• Hepatomegaly and abnormal liver function tests are common in reactive haemophagocytic syndrome (RHS).
• Liver involvement is frequent in RHS, often linked to underlying conditions.

Purpose of the Study:

• To analyze liver histology in fatal cases of reactive haemophagocytic syndrome (RHS).
• To identify characteristic histological findings of RHS in the liver and their correlation with underlying diseases.

Main Methods:

• Retrospective analysis of liver histological materials from 12 fatal RHS cases.
• Review of underlying diseases, hepatic derangements, and histological features including Kupffer cell hyperplasia and haemophagocytosis.

Main Results:

• Underlying diseases included lymphoma/leukaemia, carcinomas, infections, and drug intoxication.
• Ten patients had liver involvement by the primary disease, contributing to hepatic derangement.
• All cases showed diffuse Kupffer cell hyperplasia with haemophagocytosis; 8 had moderate to marked haemophagocytosis.

Conclusions:

• Kupffer cell hyperplasia with prominent haemophagocytosis in liver biopsies is indicative of RHS.
• These findings suggest the need for clinical monitoring in patients with suspected RHS.
• Liver biopsy findings are crucial for diagnosing RHS and understanding its pathological basis.