The
lupus anticoagulant may be accompanied by an acquired
factor II deficiency and
bleeding. We report on a patient with a
lupus anticoagulant and
factor II (Fll) deficiency responsive to
Danazol.
Acquired hypoprothrombinemia (FII) with the
lupus anticoagulant (LA) may be accompanied by a
hemorrhagic diathesis. A 64-year-old male with discoid lupus erythematosis bled after an intestinal polypectomy. His FII level was 18%, and his FII
antigen level was 20%.
Danazol (
D) (600 mg per day) administration was associated with a rise in FII activity and
antigen to 50% within 10 days. The patient underwent abdominal surgery. We studied the effect(s) of D on the FII level and on other
coagulation factors in this patient. The patient's plasma FII
antigen had a single precipitin
arc compared to the two peaks of normal plasma on counterimmunoelectrophoresis with Ca++. The samples pre- and during D
therapy had the same positively charged
arc as normal samples, although they were quantitatively different.
Neuraminidase treatment demonstrated a decrease in the positively charged migration of normal and the patient's FII
antigen. Affinity chromatography of normal and patient plasma on a
Sepharose protein A column revealed FII
antigen present in the patient's bound fraction. The relative percentages of bound FII before and during D treatment were similar. During D
therapy, levels of FIX and X rose 50-100%, and
protein C rose 20-25%, while free
protein S did not change. D is an effective
therapy for acquired FII deficiency associated with LA. D does not affect the binding of Ig to FII, but D raises FII levels by increasing synthesis of the FII
protein.