Decreased factor XIII activity during severe Henoch-Schoenlein purpura -- does it play a role?

BACKGROUND: In patients with Henoch-Schoenlein purpura (HSP), particularly with severe gastrointestinal symptoms, an associated decrease of plasma factor XIII has been observed.

PATIENT: The authors report a case of HSP in a boy and describe the development of factor XIII activities throughout the course of the disease. Every relapse of severe gastrointestinal manifestation was associated with a decrease of factor XIII. No improvement was seen after treatment with prednisone. The symptoms resolved each time factor XIII concentrate was administered. With the return of factor XIII to normal values eight weeks after admission abdominal symptoms ceased.

CONCLUSION: The documented course supports the hypothesis that factor XIII activity correlates well with the severity of abdominal symptoms. Measuring factor XIII activity helps to identify those patients with severe gastrointestinal manifestation who may benefit from substitution therapy.