Massive IgE-hyperimmunoglobulinemia and storage histiocytosis in Sézary syndrome. A postmortem study.

Substantial evidence has accumulated that the Sézary syndrome is grouped together as "cutaneous T-cell lymphoma" and forms a subset of T-cells programmed for helper interactions with B-cells in their production of immunoglobulins; IgA, IgG, and IgM. The authors describe an autopsy case of a 64-year-old Japanese man, with cutaneous T-cell lymphoma in association with IgE-type lambda hyperimmunoglobulinemia. The serologic and immunocytochemical studies suggest that Sézary cells from our patient are T-cell proliferations in association with IgE-specific helper activity. In addition, histiocytosis was interestingly noted, for the first time, in the generalized lymph nodes, spleen, and bone marrow by the autopsy. The cytoplasm of swelled histiocytes contained IgE-type lambda immunoglobulin by the PAP method that probably represented altered immunoglobulin.