Plasma Exchange in the Treatment of Patients with Systemic Amyloidosis
The demonstration of an amyloid degrading activity of human serum (Kedar et al., 1974) which appears to be defective in patients with amyloidosis led us to assess the possible benefits of plasma exchange through plasmapheresis in the therapy of advanced systemic amyloidosis. To date, five patients were enrolled in this program consisting of weekly plasmapheresis of a minimum three units of plasma which were replaced with fresh frozen plasma. One patient had primary amyloidosis, another one had rapidly progressing FAP while the other three patients had reactive systemic amyloidosis (RSA) with high SAA levels (11.2, 7.5, 8.3 µg/ml) accompanying tuberculosis, osteomyelitis, and recurrent tonsillities, respectively. 99m Technetium-diphosphonate scanning revealed amyloid deposits in the myocardium of three of these patients and in all their liver, spleen, and G.I. tract.
The exchange procedure was tolerated well by the FAP patient, who underwent 62 exchanges, but was not as well tolerated by the RSA cases who had an average of only 18.5 exchanges. The major side effects were represented by a sudden drop of their BP at the end of some runs in 7% of cases and by the development of severe allergic reactions to FFP in 2 cases. Subjective improvement was significant in the FAP case during the initial 30 exchanges and was modest in the other cases. Objectively, the 99m Tc-diphosphonate scanning showed a marked decrease of the myocardium labelling in the FAP patient and one of RSA cases after 10 exchanges. SAA level decreased by 52–65% in all three cases of RSA. Thus, it appears that plasma exchange could be of some benefit in cases of systemic amyloidosis that do not develop marked orthostatic hypotension and allergy to plasma.
KeywordsPlasma Exchange Fresh Freeze Plasma Fresh Freeze Plasma Juvenile Rheumatoid Arthritis Serum Parameter
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