Angioimmunoblastic T cell lymphoma co-occurring with gamma heavy chain disease and plasma cell dyscrasia – a case report

Background: Gamma heavy chain disease (GHCD) is a rare B-cell neoplasm that produces truncated lacks light chain-binding sites.1 Angioimmunoblastic T-cell lymphoma (AITL) associated with EBV driven lymphoproliferative disease,2 and EBV-independent plasma clonal cell expansion,3 but concurrence both has not been described. Case presentation: An 81-year-old female presented adenopathy, dysphagia B-symptoms. Immunoelectrophoresis demonstrated gamma paraprotein (16g/L) no corresponding elevated globulin (15g/L). Serum free kappa chains (κSFLC; 1334mg/L, κ/λ ratio 86.0) were by immunoturbidimetry, non-linear, previously described phenomenon in biomarker quantitation.1 Lymph node biopsy demonstrated: (1) CD4+ T cells loss of CD7 strong PD-1 expression amongst residual CD21-positive follicular dendritic networks consistent AITL; (2) Scattered B immunoblasts positive for EBER-ISH lacking chain; (3) Numerous kappa-restricted abnormal negative EBER ISH. Bone Marrow all three populations. viremia was confirmed PCR. R-miniCHOP treatment led to clinical defervescence, >1 log decrease κSFLC values, Deauville-3 metabolic response (FDG-PET). Conclusion: Herein we demonstrate AITL can co-occur GHCD EBV-negative expansion. References 1. Rajasekariah H, Jacob J, Ponraj R, et al. disease, disorder – case report literature review. Pathology 2022; 54: S77–S78. 2. Lunning MA, Julie M. Vose JM. lymphoma: the many-faced lymphoma. Blood 2017; 129: 1095–1102. 3. Balagu O, Martnez A, Colomo L, Epstein-Barr virus proliferations lymphomas peripheral lymphomas: distinctive clinicopathologic features.Am J Surg Pathol 2007; 31: 1310–1322.