Hepatitis in fatal infectious mononucleosis

Area of Science:

• Pathology
• Immunology
• Virology

Background:

• Infectious mononucleosis (IM) can lead to severe outcomes, particularly in individuals with underlying immune deficiencies.
• X-linked lymphoproliferative syndrome (XLPS) is a rare genetic disorder affecting males, predisposing them to severe complications following Epstein-Barr virus (EBV) infection.

Purpose of the Study:

• To conduct a clinicopathologic analysis of fatal infectious mononucleosis cases.
• To determine the extent and impact of hepatic dysfunction in sporadic fatal IM versus fatal IM associated with XLPS.

Main Methods:

• Clinicopathologic analysis of 30 patients with sporadic fatal IM and 31 males with fatal IM and XLPS.
• Evaluation of clinical findings, laboratory data (including heterophile antibody titers, EBV antibody studies), viral studies, histologic findings, and pedigree analysis.

Main Results:

• Hepatic dysfunction was uniformly present in both groups, leading to death in a significant proportion of cases (13/30 sporadic IM, 18/31 XLPS).
• Patients with XLPS were significantly younger (median age 2.4 yr) and had shorter survival (median 4 wk) compared to sporadic IM cases (median age 10.7 yr, median survival 8 wk).
• All XLPS patients were male, while the male to female ratio in sporadic IM was 3:2.

Conclusions:

• Hepatic dysfunction is a major cause of mortality in fatal infectious mononucleosis, irrespective of etiology.
• X-linked lymphoproliferative syndrome presents with more severe and rapid progression of fatal infectious mononucleosis, characterized by earlier onset and shorter survival.
• Early diagnosis and management strategies are crucial for improving outcomes in patients with EBV infection, especially those at risk for XLPS.