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Bleeding tendency caused by IgG inhibitor to factor XIII, treated successfully by cyclophosphamide
Author(s) -
Nakamura Satoko,
Kato Atsushi,
Sakata Yoichi,
Aoki Nobuo
Publication year - 1988
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.1365-2141.1988.tb04208.x
Subject(s) - factor xiii , prednisolone , cyclophosphamide , coagulation , medicine , c1 inhibitor , tissue transglutaminase , endocrinology , chemistry , pharmacology , immunology , gastroenterology , chemotherapy , biochemistry , enzyme , fibrinogen , angioedema
Summary A case exhibiting bleeding tendency caused by an acquired inhibitor to factor XIII is reported. The patient, a hitherto healthy 87‐year‐old Japanese man, presented with a massive subcutaneous bleeding, leading to severe anaemia. The routine coagulation study was normal except for a decreased plasma factor XIII level, which was 3% of the control level by the dansylcadaverine incorporation assay. An inhibitor of factor XIII was demonstrated to be present in the IgG fraction of the patient's plasma; by immunoblotting this inhibitor was shown to bind specifically the a and a ‘subunits of factor XIII. The IgG fraction suppressed the transglutaminase activity of activated factor XIII, but did not inhibit the molecular transformation of subunit a to a’ in the activation process. Massive infusion of plasma and factor XIII concentrate was effective for controlling the bleeding temporarily. In the long‐term prednisolone was ineffective for suppressing the plasma inhibitor level and bleeding episodes recurred. A small daily dose (50 mg) of cyclophosphamide, however, effectively decreased the inhibitor level and controlled bleeding.