Acquired C1-esterase inhibitor deficiency and recurrent herpes infection in a patient with chronic lymphocytic leukemia.
Profound hypocomplementemia was observed in a patient with chronic lymphocytic leukemia. On the basis of the results of complement measurements performed in nine serum samples taken in a period of 6 mo it was demonstrated that the patient had an acquired form of C1-esterase inhibitor deficiency: marked reduction of CH50, C1, C4, and C1-esterase inhibitor levels with normal alternative pathway, C3, and factor B activities were found. The patient had no typical attacks of angioneurotic edema; however, angioedema-like facial swelling was observed on several occasions in connection with recurrent severe herpes simplex eruptions. The possible association of the marked and long-lasting hypocomplementemia with the clinical course of the herpes infection is discussed.
D000799:Angioedema / Q000276:immunology; D003172:Complement C1 / Q000172:deficiency; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003181:Complement C4 / Q000172:deficiency; D006561:Herpes Simplex / Q000276:immunology*; D006801:Humans; D007945:Leukemia, Lymphoid / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence
D003172:Complement C1; D003174:Complement C1 Inactivator Proteins; D003181:Complement C4