Acquired C 1-inhibitor deficiency with angioedema due to pleomorphic immunocytoma in a patient with three malignant tumors: long-term follow-up data and presentation of an additional case.
Two cases of lymphoma-associated acquired C 1-inhibitor deficiency are described. In both patients, C 1-inhibitor deficiency and related symptoms preceded the diagnosis of the underlying neoplasm by several months. C 1-inhibitor deficiency was most likely due to consumption following immunocomplex formation. In both patients, a close relationship between low levels of C 1-inhibitor and tumor relapse was observed during follow-up. These findings indicate that measurement of C 1-inhibitor and complement factor C4 can be used as markers of disease activity in affected patients.
Bohle
W
W
Department of Internal Medicine, Katharinenhospital, Stuttgart, Germany.
Rüther
U
U
Bokemeyer
C
C
Nunnensiek
C
C
Müller
H A
HA
Haake
H
H
Feldmann
K
K
Jipp
P
P
D000799:Angioedema / Q000209:etiology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications*; D008137:Longitudinal Studies; D008228:Lymphoma, Non-Hodgkin / Q000150:complications*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary
D003174:Complement C1 Inactivator Proteins