Reactive and neoplastic human lymphoid cells producing J chain in the absence of immunoglobulin: evidence for the existence of 'J chain disease'?

The initiation of cytoplasmic Ig synthesis in differentiating B cells is accompanied by the start of cytoplasmic J chain production. As the cell matures further, J chain synthesis ceases (unless it is producing dimeric IgA or 19S IgM). In consequence, it is common to find Ig-positive, J chain-negative cells in reactive lymphoid tissue. However, the reverse pattern (Ig-negative/J chain-positive), which would indicate J chain production unaccompanied by Ig synthesis, has not been reported. In this paper we describe the detection of such cells in reactive human lymphoid tissue by a double immunoenzymatic labelling technique. Furthermore, retrospective immunohistological analysis of 90 cases of human high-grade lymphoma revealed three cases in which the neoplastic cells appeared to synthesize J chain but not Ig. These findings suggest that the term J chain disease might be introduced to describe this new class of lymphoid neoplasm. However, it is pointed out that immunochemical categories of human B lymphoproliferative diseases based upon patterns of Ig synthesis are often in direct conflict with histological categories (cf. mu chain-producing neoplasms) and the term J chain disease cannot therefore be recommended. It is probable that further cases of J chain-positive, Ig-negative lymphoid neoplasms, covering a range of histological categories, will be described in the future.