JILTI. 2025; 3(2): 72-74 | DOI: 10.14744/jilti.2025.87487

Wolman's Disease, Haemopagocytic Lymphohistiocytosis and Cytomegalovirus Infection: Association or Coincidence?

Sukru Gungor¹, Fatma İlknur Varol¹, Mukadder Ayse Selimoglu¹, Nese Karadag Soylu², Arzu Akyay³, Yurday Durmaz Oncul³, Emine Samdanci², Bengu Macit³, Emre Gok^1
1Department of Pediatric Gastroenterology, Inonu University, Malatya, Türkiye
²Department of Medical Pathology, Inonu University, Malatya, Türkiye
³Department of Pediatric Hematology and Oncology, Inonu University, Malatya, Türkiye

Wolman's disease, an infantile form of lysosomal acid lipase deficiency, is very rare and usually fatal in infancy. In this article we present two infants with Wolman's disease associated with cytomegalovirus infection, one with surrenal calcifications and the other with histopathological liver calcifications and haemophagocytic lymphohistiocytosis.
Both cases had complaints of vomiting and fever for two months. A family history of consanguinity and infant deaths of unknown cause was reported. Physical examination revealed jaundice and hepatosplenomegaly. Cytomegalovirus infection, unresponsive to ganciclovir treatment, was diagnosed in both cases. While haemophagocytic lymphohistiocytosis and histopathological calcifications in the liver were observed in the first case, classical surrenal calcifications were seen in the second case.
It is not clear whether the presence of cytomegalovirus infection in both cases and haemophagocytic lymphohistiocytosis in one case are coincidental. Furthermore, histopathological calcification in the liver appears to be a diagnostic criterion, as is adrenal calcification, although it has never been reported before.

Keywords: Hemophagocytic Lymphohistiocytosis, Cytomegalovirus Infection, Wolman's Disease

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