Plasmapheresis in the dysproteinemias.
The dysproteinemias consist of a broad range of serious disease states with the common thread of excessive production of an abnormal, or para-protein. Various clinical syndromes may arise, either from the underlying disease process, the excess paraprotein, or both. Clinical presentation depends upon the organ system(s) affected by the abnormal protein. Diseases included under the classification of dysproteinemias include cryoprotein-related diseases, Waldenström's macroglobulinemia, hyperviscosity syndrome, monoclonal gammopathy, multiple myeloma, light chain disease, and amyloidosis. Plasmapheresis, often in conjunction with other therapies, has been widely used to treat the dysproteinemias and their resulting clinical syndromes. Automated plasmapheresis, which separates plasma from the cellular blood elements by centrifugation, is used most commonly in the United States. Membrane separation and immunoadsorption techniques are more commonly used in Europe and Japan. In automated plasmapheresis, the plasma is removed from the patient's circulation and replaced with a protein-based fluid such as 5% human albumin solution or plasma protein fraction or with fresh frozen plasma. Membrane separation and immunoadsorption allow the offending proteins to be removed more selectively from the patient's plasma prior to the plasma being returned to the patient. This review article presents a description of each disease, the rationale for plasmapheresis therapy, recommended schedules of plasmapheresis, and the use of adjunctive therapies. Results of published studies, case reports, and the author's experience in treating these diseases will serve as the foundation for discussion.
Drew
Mary Jo
MJ
Transfusion medicine, Henry Ford Hospital Blood Bank, Detroit, Michigan 48202, USA.
D000686:Amyloidosis / Q000628:therapy; D001796:Blood Protein Disorders / Q000628:therapy*; D002498:Centrifugation; D003449:Cryoglobulinemia / Q000628:therapy; D006801:Humans; D009101:Multiple Myeloma / Q000628:therapy; D010265:Paraproteinemias / Q000628:therapy; D010956:Plasmapheresis; D008258:Waldenstrom Macroglobulinemia / Q000628:therapy