A 72-year-old female presented with multiple lung and brain lesions with no lymphadenopathy and no renal disease. A lung wedge biopsy was performed and sent for histopathological examination.
Final Diagnosis: Lymphomatoid Granulomatosis; Low Grade
The lung biopsy shows diffuse parenchymal and angiocentric CD4-T-cell predominant lymphocytic proliferation with mild to moderate cytological atypia and back ground poorly-formed granulomatous inflammation. EBV positive large B-cells are scattered and few (<5%). Similar features were described in a prior right frontal brain lesion, indicating involvement by the same process. Extensive infectious disease work up to exclude bacterial, fungal and viral infection were performed and all are negative. These studies include HTLV1I/II serology, microbiology culture, among others. Additionally, ANCA associated studies for vasculitis are performed and are negative. TCR gamma gene rearrangement PCR test showed a non-clonal pattern. Lymphomatoid granulomatosis (LYG) is a rare EBV associated lymphoproliferative disorder that is characterized by sparse EBV positive B-cells and minimal or absent coagulative necrosis; as in this case.
Reference:
- Ref: Blood (2020) 135 (16): 1344–1352
This case is contributed by Drs. Saja Asakrah and Zaid Mahdi, Emory University Hospital.