Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone.
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.
D001856:Bone Marrow Examination; D002850:Chromatography, Gel; D003176:Complement C3 / Q000032:analysis; D003298:Coombs Test; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004591:Electrophoresis, Polyacrylamide Gel; D004592:Electrophoresis, Starch Gel; D006362:Heavy Chain Disease / Q000188:drug therapy*; D006801:Humans; D007106:Immune Sera; D007122:Immunoelectrophoresis; D007128:Immunoglobulin Fragments; D008198:Lymph Nodes / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*; D012075:Remission, Spontaneous; D012867:Skin / Q000648:ultrastructure; D012967:Sodium Dodecyl Sulfate; D014652:Vascular Diseases / Q000473:pathology
D003176:Complement C3; D007106:Immune Sera; D007128:Immunoglobulin Fragments; D012967:Sodium Dodecyl Sulfate; D003520:Cyclophosphamide; D011241:Prednisone