A progressive immunoproliferative disease consisting of a haemorrhagic state characterized by elevated serum globulin fraction, due to the presence of globulins of abnormally high molecular weight, adenopathy, and bone marrow changes. Hemorrhagic diathesis is the principal symptom of the disorder, with bleeding from the nose, eyes, and mouth, and, sometimes, the vagina and gastrointestinal tract. Early symptoms include fatigue, pallor, loss of weight, anemia, and general malaise. The first complaint is often visual trouble. The syndrome occurs chiefly in males over 50 years of age, but it has also been seen in the female and younger adults.
Waldenström in 1948 described this condition in six patients and applied the term "macro-globulinemia."
- J. G. Waldenström:
Incipient myelomatosis or "essential” hypergloulinemia with fibrinogenopenia - a new syndrome?
Acta Medica Scandinavica, Stockholm, 1944, 117: 216-247.