Viral associated haemophagocytic syndrome — A post mortem diagnosis

Abstract

The clinical and autopsy findings in a young serving soldier, who was hospitalised as a case of pyrexia of unknown origin with hepatosplenomegaly, are presented. Unfortunately, the final diagnosis of viral associated haemophagocytic syndrome (VAHS) could only be established after autopsy. VAHS is a benign disorder characterized by systemic proliferation of haemophagocytic histiocyte and its precursors [1]. It is commonly associated with acute systemic viral infections like Epstein-Barr, Cytomegalovirus, Herpes simplex, Varicella zoster, Adeno and HIV viruses [2, 3, 4]. Initially, Risdall et al in 1979 [5], in their original report found the association of these cases with viral infections but in their later study of the cases, no such proven association could be established [3]. This case of VAHS is reported because of its rarity and difficulties in establishing the diagnosis by unwary clinicians. Secondly, in addition to wide spread proliferation of haemophagocytic histiocytes in various organs, the lymph nodes showed extensive necrosis with marked lymphoid depletion, which is an uncommon finding [4].