A unique case of kappa light-chain disease associated with cryoglobulinemia, pyroglobulinemia, and hyperviscosity syndrome

Abstract

Monoclonal gammopathies (paraproteinemias) occur in the settings of Waldenstrom’s macroglobulinemia, plasma cell dyscrasias, and primary lymphoproliferative diseases, as well as in several unrelated entities such as rheumatoid arthritis and chronic infections. Although serum hyperviscosity may occur with any of these disorders, and does occur in 4% of cases of multiple myeloma [l], it has been reported only once in relation to a light-chain myeloma variant but without the accompanying clinical complications [2]. The syndrome often associated with serum hyperviscosity may include any of all of the following manifestations: ocular, neurologic, hemorrhagic, and cardiovascular. Therapy for this disorder usually involves supportive treatment and control of the underlying disease. Measures such as plasmapheresis are designed to reduce the serum concentration of the homogeneous protein and thus the serum viscosity. Zinneman [3] defines a combined cryo-pyroglobulin as an abnormal immunoglobulin molecule that forms both a reversible cryoprecipitate at low temperatures and an irreversible pyrogel at elevated temperatures. This is differentiated from a Bence Jones protein, which will redissolve if heating is continued above 56°C. Only one case of a light-chain protein meeting the criteria for a combined cryo-pyroglobulin [4], but without serum hyperviscosity, has been reported. In fact, only one case of pyroglobulin formation and a small number of cases of cryoglobulin formation have been seen in relationship to light chains [4-91. The molecular mechanisms responsible for these temperature-sensitive precipitates have been discussed [3]. We report the unique instance of a patient with kappa light-chain myeloma whose clinical condition showed a dramatic response to therapy. His light chain was also remarkable for its combined cryo-pyroglobulin properties.