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

Abstract:

: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.

journal_name

Blood

journal_title

Blood

authors

Lyons RM,Chaplin H,Tillack TW,Majerus PW

subject

Has Abstract

pub_date

1975-07-01 00:00:00

pages

1-9

issue

1

eissn

0006-4971

issn

1528-0020

journal_volume

46

pub_type

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