Alpha-2-antiplasmin deficiency is a rare autosomal recessive condition characterized by increased fibrinolysis which can subsequently lead to uncontrolled hemorrhage. First described in Japan in 1969 by Masateru Kohakura, alpha-2-AP deficiency is rare with fewer than 50 published case studies to date. Individuals with alpha-2-AP deficiency will have normal coagulation parameters including platelet count, bleeding time, Prothrombin Time (PT), activated Partial Thromboplastin Time (aPTT), and clotting factor titers. In alpha-2-AP deficiency patients, clots are able to form normally but degrade in an accelerated fashion prior to the initiation of tissue and vascular repair. Diagnosis of alpha-2-AP deficiency thus requires a high index of suspicion and can be confirmed with specific functional and immunological antiplasmin assays. Here we present a case of a 67-year-old male with known history of homozygous alpha-2-AP deficiency undergoing intramedullary fixation of a distal femur fracture, followed by a discussion of the literature on Perioperative and postoperative strategies for individuals with α2-AP deficiency.
A 67-year-old male with known history of homozygous alpha-2-AP deficiency presented to our institution with a displaced distal left femur fracture status post a fall from standing height. He has a past medical history of coal workers pneumoconiosis, hypertension, treated hepatitis C infection, and hypothyroidism as well as prior right humeral fracture requiring surgical correction and previous intracranial hemorrhage in 2013 from a horse accident, both successfully managed with antifibrinolyitic therapy. Immediate pre-operative management for bleeding risk included infusion of FFP and 1 gram of tranexamic acid IV bolus. He then received a continuous infusion of tranexamic acid intraoperatively. He underwent uneventful intramedullary fixation of the left distal femur. After completion of the infusion, he was placed on tranexamic acid 1300 mg q 8 hours. Unfortunately, on post-operative day 7, patient developed a new Left Lower extremity deep venous thrombosis despite prophylactic LMWH.
Patients with alpha-2-AP much like those with hemophilia have increased bleeding tendencies during surgical procedures. Generally, adequate hemostasis can be managed with administration of anti-fibrinolytic agents. As in our case, there remains the potential for thrombotic events related to antifibrinolyitic therapy and the benefits versus risks of treatment will need to be considered in every surgical case in patients with diagnosis of homozygous alpha-2-AP deficiency. Since it is a rare entity, there is limited medical literature to utilize as guidelines in managing such patients and the approach to post-operative hemorrhaging and DVT prophylaxis in individuals with defects in the fibrinolysis pathway should be considered on a case-by-case basis taking into consideration additional risk factors for both bleeding and thrombosis.
Citation: Nakazawa M, Monohan G (2019) Perioperative and Postoperative Management in Patients with Alpha-2-Antiplasmin Deficiency: A Case Study of 67-Year-Old Male Undergoing Operative Distal Femur Repair. J Hematol Blood Transfus Disord 6: 023.
Copyright: © 2019 Mary Nakazawa, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.