Two related cases of primary complement deficiency

Abolhassan Farhoudi, A Farhoudi, Nasrin Bazargan, N Bazargan, Z Pourpak, Zahra Pourpak, Mahdi Mahmoudi, M Mahmoudi
Immunological investigations · 2003 · vol. 32(4) , pp. 313–321 · doi:10.1081/imm-120025108 · PMID:14603997 · W1965261400
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Two related patients presented with primary complement deficiencies: one with recurrent pneumococcal meningitis and C3 deficiency, and another with hereditary angioedema and C1INH deficiency.

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Abstract

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 pneumococcal 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 normal. This patient now takes prophylactic antibiotics and the meningitis had not recurred, but he does have glomerulonephritis. 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 which is very low compared with normal healthy subjects (C1INH was 40-50 mg/dl) 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 without a family background. The condition may be due to genetic mutation. The angioedema was controlled with Danazol and Stanasol. As our patients are related, this may suggest a genetic relationship between these two disorders.

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MeSH descriptors

Complement C3 Adult Anti-Bacterial Agents Anti-Bacterial Agents Complement C3 Danazol Danazol Dyspnea Dyspnea Edema Edema Edema Female Genetic Diseases, Inborn Genetic Diseases, Inborn Genetic Diseases, Inborn Genetic Diseases, Inborn Glomerulonephritis Glomerulonephritis Humans

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