title: 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.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Katharinenhospital, Stuttgart, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Bohle
authors.forename: U
authors.initials: U
authors.lastname: Rüther
authors.forename: C
authors.initials: C
authors.lastname: Bokemeyer
authors.forename: C
authors.initials: C
authors.lastname: Nunnensiek
authors.forename: H A
authors.initials: HA
authors.lastname: Müller
authors.forename: H
authors.initials: H
authors.lastname: Haake
authors.forename: K
authors.initials: K
authors.lastname: Feldmann
authors.forename: P
authors.initials: P
authors.lastname: Jipp
mesh_terms: 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
chemical_list: D003174:Complement C1 Inactivator Proteins