Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 2 2 2003 06 15 69 74 A. Farhoudi Nasrin Bazargan Zaiirn Pourpak Maryam Mahmoudi 2015 10 01 Primary complement deficiencies are rare and two related patients are reported here. The first patient is a 41- year- old man with eighteen episodes of pneumo¬coccal meningitis and other purulent infections. The serum C3 level was checked at three separate times, showing that this was a primary C3 deficient case; other immunological tests were however normal. This patient now takes prophylactic antibiotics and the meningitis has not recurred, but he does have glomerulone¬phritis. The second case is a 40 - year-old woman with repeated episodes of orofacial and laryngeal edema and dyspnea. The serum C1INH levels were 4.3 to 7 mg/dL which were very low compared with normal healthy subjects (C,INH was 40-50 mg/dL in ten normal controls) and C4 was lower than normal but other immunological tests were normal. Other causes of angioedema such as lymphoproliferative disorders were excluded. She had hereditary angioedema with¬out a family background. The condition may be due to genetic mutation. The angioedema was controlled with Danazol and Stanasol. As our patients are re¬lated, this may suggest a genetic relationship between these two disorders. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/36 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/36/36