title: Angiocentric lymphomatoid granulomatosis and severe hypogammaglobulinaemia.
abstract: Angiocentric lymphomatoid granulomatosis is a rare lymphoproliferative disease, mainly associated with pulmonary manifestation. Its origin is unknown, but Epstein-Barr virus may be one of the etiological factors. A 51-year-old male had an abdominal laparotomy in 1994 and a large granulomatous mass was removed from behind the cecum. No specific therapy was administered. In February 1998 multiple pulmonary lesions were found by X-ray and thoracoscopic biopsy was made. The histopathological diagnosis was angiocentric lymphomatoid granulomatosis. The patient received 6 cycles of CHOP chemotherapy, with which a complete remission was achieved. A consistent severe hypogammaglobulinaemia was detected, so the diagnosis of common variable immunodeficiency (CVID) was established. The diagnosed CVID was the probable causative factor of the angiocentric lymphomatoid granulomatosis. After the CHOP treatment, the patient is on intravenous immunoglobulin substitution and is well up to today.
authors.affiliation: Third Department of Medicine, Medical and Health Science Centre, University of Debrecen, Móricz Zs. u. 22., 4004 Debrecen, Hungary. varoczy@iiibel.dote.hu
authors.forename: László
authors.initials: L
authors.lastname: Váróczy
authors.forename: Lajos
authors.initials: L
authors.lastname: Gergely
authors.forename: Szabolcs
authors.initials: S
authors.lastname: Szakáll
authors.forename: Arpád
authors.initials: A
authors.lastname: Illés
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000628:therapy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008230:Lymphomatoid Granulomatosis / Q000150:complications* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D016756:Immunoglobulins, Intravenous; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone