General description

Lymphomatoid granulomatosis (LYG) is an Epstein-Barr virus-associated B-cell lymphoproliferative disorder characterized by angio-centric and angio-destructive growth of atypical lymphoid cells. Granulomas are not formed. It is more common in males, typically affecting individuals in their 30s-50s, though it can occur at any age. A significant proportion (10-60%) of patients eventually develop malignant lymphoma.

The most commonly affected organs include the lungs (90%), skin (25-50%), kidneys (30-40%), liver (29%), and central nervous system (26%). The lungs are the most common site of involvement, with the majority of patients initially presenting with pulmonary symptoms. Lymph node enlargement is rare and should raise suspicion for malignant lymphoma. Skin manifestations are the second most frequent.

The central nervous system is affected in approximately one-third of cases, often presenting with cranial nerve symptoms, hemiparesis, or altered mental status. While extra-pulmonary involvement often includes the skin and CNS, isolated CNS LYG is rare.