A Case with SLE Showing Acquired Low Plasma F.XIII Level Followed by Severe Hematoma Characteristic of F.XIII Deficiency

Abstract

Although the plasma F.XIII Levels were lowered in some SLE patients, we experienced one case of a 20 year-old female with SLE, showing an acquired low F.XIII level (20%) followed by the severe and prolonged oozing characteristic of F.XIII deficiency. The patient had been diagnosed 3 years earlier as SLE with thrombocytopenia, and a therapy including prednisolone (PSL) was begun. At that time, she suffered a rectal perforation, which was successfully operated. On Apr. 25, 1987, when she was admitted to our hospital for the fourth time, she complained of proteinuria, the laboratory findings on liver function and hemostatis being all within normal range. After four months of therapy including PSL, subcutaneous bleeding appeared, which rapidly grew into a huge hematoma covering her back. At this time, too, PT, APTT, Fbg, AT III, and each clotting factor activity were within normal range, except for a low platelet count (92,000/μl) and a low F.XIII level (20%). At least, obvious DIC could be denied. The administration of an F.XIII concentrated product (Fibrogammin) for five days resulted in partial improvement of her huge hematoma and exfoliative wound. Nevertheless, 4 days thereafter obvious DIC occurred resulting in her death. The decrement of F.XIII in this SLE patient may be attributable to pre-DIC or may reflect latent vascular injury due to SLE. The monitoring of plasma F.XIII in SLE patients is very important in order to avoid and to treat hemorrhagic complications.