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Cryo- and macroglobulinemia

1956; Elsevier BV; Volume: 20; Issue: 4 Linguagem: Inglês

10.1016/0002-9343(56)90139-5

1555-7162

Ian R. Mackay, Nils Eriksen, Arno G. Motulsky, Wade Volwiler,

Erythrocyte Function and Pathophysiology

Five patients with varying degrees of cryo- and macroglobulinemia have been studied and the physicochemical characteristics of these abnormal proteins investigated. 1.1. Macroglobulins are giant protein molecules with a molecular weight of over one million. Cryoglobulins are proteins which precipitate on cooling but redissolve on warming to body temperature. Occasionally, an abnormal plasma protein may be both a cryo- and macroglobulin. These abnormal proteins are probably elaborated by the reticuloendothelial-plasmacyte system. 2.2. The electrophoretic mobility of cryo- and macroglobulins ranged between that of beta and gamma globulins. Electrophoretic patterns of severe macroglobulinemia are indistinguishable from those of myelomatosis. 3.3. Ultracentrifugal analysis is required for the detection of macroglobulins (S constant greater than 15). Multiple macroglobulin components with differing sedimentation constants were found. Macroglobulinemia is not present in myelomatosis. 4.4. The physicochemical characteristics of these proteins were striking in their variability. 5.5. A “protein-cryocrit” determination was devised for rapid rough quantitation of cryoglobulins. 6.6. The water-dilution screening test (Sia or Brahmachari test) was found unreliable for detecting macroglobulins. 7.7. Clinically, cryoglobulins may be causally related to Raynaud's syndrome and cold purpura. However, no regular relationship could be found between the amount of cryoglobulins and the presence or severity of such symptoms. 8.8. Cryo- and macroglobulinemia is not specific for any disease entity but cryoglobulins are most frequently seen in myelomatosis. Marked macroglobulinernia is frequently associated with a definite clinical syndrome classified at present as “Macroglobulinemia of Waldenström” with dyspnea, mucous membrane bleeding and bone marrow infiltration of small atypical lymphocytic cells. Immunologically, these macroglobulins may be distinct. 9.9. The literature of cryo- and macroglobulinemia is comprehensively surveyed and discussed. The pathologic physiology of cryo- and macroglobulinemia is portrayed diagrammatically.