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Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone

RM Lyons, H Chaplin, TW Tillack and PW Majerus

A patient, CAL, with gamma heavy chain disease is presented who has had a complete remission lasting over 2 yr with combination chemotherapy consisting of pulsatile cyclophosphamide and prednisone. The patient exhibited many features of an atuoimmune process including a vasculitis, low serum complement levels, a positive antiglobulin (Coombs) test, Raynaud's phenomenon, and keratoconjunctivitis sicca. The CAL paraprotein was found to have several previously undescribed characteristics. It reacted with antisera to Fd, Fab, and Fab', suggesting that most of the Fd portion of the molecule was intace. CAL protein consists of two polypeptide chains of molecular weight 49,000 covalently linked to form a dimer of 95,000 molecular weight. The covalent linkage suggests that the hinge region of this gamma heavy chain is intact.

Volume 46, Issue 1, pp. 1-9, 07/01/1975
Copyright © 1975 by The American Society of Hematology


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  Copyright © 1975 by American Society of Hematology         Online ISSN: 1528-0020