Pathological studies on a long-term survived case of gamma heavy chain disease--a brief review of 30 reported cases and a proposal for histological typing.

A pathological and brief clinical study on the eleventh case of gamma heavy chain (gamma-chain) disease who died at the age of 44 after a long course of 12 years from the assumed onset of the disease was presented. Clinicopathological observations of the case showed a neoplastic nature which caused her death complicated by asthmatic attacks. Autopsy findings were characterized by diffuse infiltration of lymphoplasmacytoid cells and a few large immunoblastic cells into various organs. Literatures of 30 cases reported in the past, and the modern concept of lymphoma strongly suggest that the classificatory position of gamma-chain disease as well as Waldenström's macroglobulinemia should be placed between multiple myeloma and classic malignant lymphoma as an independent disease entity belonging to the same category. A proposal of histological typing of the disease was made in order to simplify various diagnostic designations in the literatures:--gamma-chain disease, 1) reticular type, 2) lymphocytic predominance, 3) plasmacytic predominance, 4) lymphoplasmacytoid cell type, 5) immunoblastic type. The present case belongs to type 4.