Evidence That the Malignant Lymphoma of Sjögren's Syndrome Is a Monoclonal B-Cell Neoplasm

TLDR

The benign lymphoepithelial salivary‑gland lesions preceding these malignant tumors contained roughly equal numbers of kappa‑ and lambda‑light‑chain–positive lymphocytes. The study aimed to characterize the cellular features of malignant lymphomas that developed in Sjögren’s syndrome patients and their antecedent benign salivary‑gland lesions. An immunoperoxidase technique was employed to detect intracellular gamma, alpha, and mu heavy chains as well as kappa and lambda light chains. Six of nine lymphomas were monoclonal IgMK B‑cell tumors, demonstrating a progression from polyclonal infiltrates to monoclonal neoplasms in Sjögren’s syndrome.

Abstract

We studied the malignant lymphomas that developed in patients with Sjögren's syndrome and the antecedent benign salivary-gland lesions to determine their cellular characteristics. We used an immunoperoxidase technic that identified intracellular gamma, alpha and mu heavy chains and kappa and lambda light chains. In six of nine patients, the lymphomas were composed of cells containing intracytoplasmic immunoglobulin that was exclusively IgMK. The benign lymphoepithelial salivary-gland lesions preceding these malignant tumors consisted of approximately equal numbers of lymphoid cells containing either kappa or lambda light chains. Thus, in some patients with Sjögren's syndrome, there may be a progression in the lympho-proliferative lesions from a polyclonal infiltrate to a monoclonal neoplasm. Intracytoplasmic immunoglobulin identifies six of the nine cases as being B-cell in origin.