Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
A Case of Secondary Cì Inhibitor Deficiency Associated with Benign Monoclonal Gammopathy and Angioneurotic Edema
Motoharu KONDONobuyoshi YOKOEHidetsugu NISHIBORIShuhei TAKEMURAToshikazu YOSHIKAWAHaruki KATOKeimei HOSOKAWATatsuo ABE
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1978 Volume 19 Issue 11 Pages 1581-1587

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Abstract

A 68 year-old female was admitted to our hospital on September, 1976, with a history of recurrent episodes of abdominal pain and angioneurotic edema of the skin, pharynx and larynx since the age of 62. She had no family history of the same symptoms. Both hematological and immunological studies revealed the presence of benign monoclonal gammopathy. Serum complement level CH 50 and complement components C1, C4, C2 and C ?? INH in the patient's serum were found to decrease, but the sera of her son and grandson had normal complement.
After admission she had suffered from episodes of typical angioneurotic edema 2 or 3 times a month. By the administration of t-AMCHA (Transamin), these symptoms disappeared for 50 days. Since the episodes of angioneurotic edema recurred, she was treated with an anabolic steroid (Anadrol). No episode of angioneurotic edema or abdominal pain reappeared as C ?? INH level increased.

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© 1978 The Japanese Society of Hematology
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