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Amyloidosis Associated with the Nephrotic Syndrome and Transfusion Reactions in a Haemophiliac
Author(s) -
Prentice C. R. M.,
Izatt Marian M.,
Adams J. F.,
McNicol G. P.,
Douglas A. S.
Publication year - 1971
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1971.tb03442.x
Subject(s) - nephrotic syndrome , medicine , antibody , amyloidosis , serology , immunology , renal function
S ummary . It is suggested that the immunological response in a severe haemophiliac to longstanding incompatible plasma infusions may have been responsible for the development of amyloidosis which culminated in the nephrotic syndrome. Serological studies showed that the patient had a circulating anti‐Gm(1) antibody, and also that possibly he was on occasions reacting violently towards the infusion of immune complexes formed between the IgG1 (Fc region) antigens, Gm(1) and ISf(1) and their antibodies. Despite the fact that there was a marked reduction in the number and severity of transfusion reactions after treatment solely with selected Gm(‐1) plasmas, there was a progressive deterioration in the patient's renal function.