title: [Angioedema: first manifestation of non-Hodgkin's lymphoma].
abstract: Angioedema can be hereditary or acquired. Hereditary angioedema is a genetic disease transmitted with an autosomal dominant mechanism. Acquired angioedema usually occurs after the second decade of life and is often related to an underlying disease. In a 48-year-old male patient a diagnosis of a non-Hodgkin lymphoma was made after two episodes of angioedema. No previously documented cases of angioedema in his family were reported; physical examination and routine blood samples were normal whereas the serum levels of C1-esterase inhibitor were reduced. Three months later he presented with epigastric pain after meals and weight loss. Abdominal ultrasonography and computed tomography showed two solid splenic masses infiltrating the greater curvature of the stomach and a 2 cm aortic lymph node. Digestive endoscopy confirmed the neoplastic infiltration. A diagnosis of anaplastic large-cells lymphoma CD30+, anaplastic lymphoma kinase negative was made. The disappearance of the neoplastic gastric infiltration and the decrease in size of the aortic lymph node and splenic mass were achieved after chemotherapy. Serum levels of C1-esterase inhibitor returned to normal. An adult onset not associated with a family history of angioedema should lead the physician to suspect the presence of a major disease.
authors.affiliation: Divisione di Clinica Medica Generale e Terapia Medica Luigi Condorelli, Dipartimento di Medicina Interna e Patologie Sistemiche, Università degli Studi di Catania. rosfoti@tiscalinet.it
authors.forename: Rosario
authors.initials: R
authors.lastname: Foti
authors.forename: Paolo
authors.initials: P
authors.lastname: Fazio
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lizzio
authors.forename: Roberto
authors.initials: R
authors.lastname: Leonardi
mesh_terms: D000799:Angioedema / Q000209:etiology* / Q000276:immunology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology* / Q000276:immunology; D014408:Biomarkers, Tumor / Q000097:blood; D001761:Bleomycin / Q000008:administration & dosage; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000276:immunology*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000032:analysis; D011241:Prednisone / Q000008:administration & dosage; D013154:Spleen / Q000473:pathology; D013270:Stomach / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D001323:Autoantibodies; D014408:Biomarkers, Tumor; D003174:Complement C1 Inactivator Proteins; D009363:Neoplasm Proteins; D001761:Bleomycin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone