A 65-year-old male underwent vascular repair for a psuedoaneurysm of left brachiocephalic arteriovenous fistula. During surgery he was noted to have excessive bleeding and also postoperatively, he continued to ooze from the surgical site. His labs revealed PT of 33.5 seconds, INR of 3.3, and PTT of 53.7 seconds. His mixing studies showed no correction of PTT or PT after addition of serum suggesting presence of an autoantibody. His thromboplastin-inhibition assay and hexagonal-lipid neutralization test were positive indicating lupus anticoagulant (LA) as the most likely cause. He also had mildly decreased levels of factors XI, IX, and V with normal levels of other factors. His rheumatological panel didn’t reveal presence of any auto-antibodies to the common autoimmune diseases. LA hypoprothrombinemia syndrome (LAHPS) was deemed to be the most likely cause of the condition. He was initially treated with high dose steroids, blood product replacements, and replacement factors but unfortunately, he wasn’t aggressively treated as per family’s wishes and he gradually succumbed to death.
Discussion:
Lupus anti-coagulant is an antibody directed against plasma proteins that binds to the phospholipids of the coagulation cascade. The clinical picture of LA disorders encompasses both ends of the coagulopathy spectrum but predominantly presents as a pro-thrombotic condition with most cases diagnosed during work up of deep venous thrombosis, pulmonary embolism, or spontaneous abortions. LA can cause bleeding in rare situations if the patient has hypoprothombinemia as shown in the figure attached. This entity is known as LAHPS. Isolated LAHPS is an extremely rare condition with only a handful of cases reported. LAHPS is commonly associated with systemic lupus erythematosus and has been reported in young females. It can cause life-threatening hemorrhages and thus being cognizant of this rare entity is crucial. High-dose steroids are considered the first-line therapy and there is debate about second-line and maintenance therapy. Potentially, plasma exchange and immunosuppressant therapy can be used but further research is warranted in this area. To the best of our knowledge, this is the first reported case of LAHPS in an elderly (age>60) male.
Conclusions:
LAHPS is an extremely rare condition which presents as bleeding in the presence of lupus anticoagulant as opposed to common presentation with clots. It is usually seen in young females with SLE but should be considered in any age or sex group.