Lymphocytic lymphoma and acquired C1 esterase inhibitor deficiency.
We report a 59-year-old woman who had progressive symptoms of angioedema for 3 years, culminating in an attack of laryngeal stridor. C1 esterase inhibitor deficiency was confirmed and treatment with tranexamic acid produced considerable clinical benefit and improvement in complement levels. Two years later she developed clinical evidence of lymphocytic lymphoma. Splenectomy resulted in rapid correction of complement abnormalities. The patient received 6 months of chemotherapy after surgery and remained asymptomatic with normal complement levels 4 years later.
Richardson
S G
SG
Department of Medicine, Russells Hall Hospital, Dudley, West Midlands, U.K.
Clarke
C W
CW
Gasson
G B
GB
D000799:Angioedema / Q000150:complications / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D008875:Middle Aged; D014148:Tranexamic Acid / Q000627:therapeutic use
D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4; D014148:Tranexamic Acid