Bleeding tendency caused by IgG inhibitor to factor XIII, treated successfully by cyclophosphamide.

Abstract

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.

• Authors: S Nakamura, A Kato, Y Sakata, N Aoki
• Journal: British journal of haematology (Br J Haematol) Vol. 68 Issue 3 Pg. 313-9 (Mar 1988) ISSN: 0007-1048 [Print] England
• PMID: 3258529 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulin G
• Factor VIIIa
• Cyclophosphamide
• Factor XIII

Topics

• Aged
• Cyclophosphamide (therapeutic use)
• Factor VIIIa
• Factor XIII (antagonists & inhibitors, metabolism)
• Hemorrhagic Disorders (drug therapy)
• Humans
• Immunoglobulin G (immunology, metabolism)
• Male