Abstract
Forty patients with Waldenström’s macroglobulinemia were evaluated for criteria of diagnosis, clinical presentation and course, response to therapy, and appropriateness of the classification primary or secondary. A wide spectrum of presentations, course, and complications were present. Thus, the major criteria for diagnosis was a monoclonal IgM serum protein abnormality present in concentrations greater than 1 g/100 ml. Clinical manifestations included weight loss, mucous membrane bleeding, presence of abnormal masses, lymphadenopathy, hepatosplenomegaly, peripheral neuropathy, and central nervous system abnormalities. A high incidence (60%) of associated malignancy was noted. The hyperviscosity syndrome was a frequent complication (33%). Alkylating drugs were successful in inducing remission in approximately 40% of the patients. Plasmapheresis was uniformly successful in relieving hyperviscosity. It was found that attempts to classify patients as primary or secondary frequently proved in error with long-term observation and as such should be discarded. Hypotheses of etiology are discussed and include the IgM proteins as: markers of a malignant lymphoid line, antibodies to microscopic or submicroscopic organisms related (or not related) to tumor induction, and antibodies to tumor-specific antigens. Firm evidence for any of these possibilities is not available.
