Angioimmunoblastic T-cell lymphoma diagnosed from pleural fluid by integration of morphologic, immunophenotypic, and molecular findings.

An 88-year-old man with end-stage renal disease on hemodialysis presented with shortness of breath and was found to have lower extremity edema and bilateral pleural effusions on a chest X-ray. A therapeutic and diagnostic thoracentesis was performed, and cytologic examination revealed atypical mononuclear cells. Based on this, flow cytometry was performed on the pleural fluid, along with immunostains on the cellblock and a next-generation sequencing (NGS) panel. A definitive diagnosis of angioimmunoblastic T-cell lymphoma (AITL) was made based on demonstrating an atypical T follicular helper cell population expressing CD10, BCL6, CXCL13, CD200, CD57, and PD1, and detection of pathogenic variants in RHOA, IDH2, and TET2. This case represents the first reported case where a primary diagnosis of AITL was made on a body fluid specimen and highlights how immunophenotyping and NGS can provide a definitive diagnosis of AITL on a cytologic specimen.