The entity known as macroglobulinemia was first described in 1944 by Jan Waldenström.1 Most of the articles referring to this condition, with a few notable exceptions, describe the ocular findings briefly. Many of the descriptions are at variance, but certain fundus changes occur frequently enough to suggest the diagnosis of macroglobulinemia.
Macroglobulinemia may be defined as the presence of abnormal amounts of an abnormally high molecular weight globulin in the plasma. The normal γ-globulins have a molecular weight of approximately 150,000; in macroglobulinemia, molecular weights over one million have been recorded.2 It is a rare disease occurring primarily in males over the age of 50.
Symptoms may consist of malaise, fatigue, weight loss, bleeding tendencies (especially from the gums and nasal mucosa), and visual depression of varying degree. Central nervous system signs such as aphasia and hemiparesis have been recorded.3
Physical findings include lymphadenopathy, hepatosplenomegaly, purpura, and
COYLE JT, FRANK PE, LEONARD AL, WEINER A. Macroglobulinemia and Its Effect upon the Eye. Arch Ophthalmol. 1961;65(1):75-80. doi:10.1001/archopht.1961.01840020077015