Two Consecutive Invasive Surgeries Utilizing Zymogen Protein C (ZPC) That Enhanced Patient Safety and Reduced Costs.

This case report describes a major surgical procedure for a protein C-deficient, hypercoagulable patient who underwent two back-to-back invasive surgeries, hip replacement, and spinal stenosis correction. The patient, an 84-year-old male with a history of deep vein thromboses (DVT) and pulmonary emboli (PE), was treated pre-, peri-, and postoperatively with zymogen protein C (ZPC-Baxter, International) and recovered without clotting or increased bleeding. During the procedure, the patient was not administered any other anticoagulants. There have now been several case reports on different patients with unrelated teams in various locations worldwide using zymogen protein C during surgical procedures. Thus, this procedure is becoming a viable choice for patients with a high probability of clotting during and after invasive surgery. This case focuses on accomplishing safer surgery and reducing costs, by using less ZPC while accomplishing two surgeries in one procedure. As a result, this procedure might be useful for many medical situations where acquired protein C deficiency could be a problem (e.g., sepsis, pregnancy, etc.). This approach may have greater application to medical conditions other than protein C deficiency, where clotting and inflammation can become issues.

A Compelling Case for the Use of Perioperative Zymogen Protein C for Increased Patient Safety.

It is imperative to maintain normal blood flow to provide adequate oxygen supply to specific organs and cells, as well as for the removal of metabolic byproducts. Therefore, any situation that results in blood clotting can injure or kill living tissues. In this paper, we describe a case where a protein C deficient subject who would, by all medical indicators, be at 100 % risk of experiencing thrombophlebitis, deep vein thrombosis, and or lung emboli, is able to escape all pathologies by using perioperative zymogen protein C (ZPC). This protein C deficient patient has a long history of blood clotting, particularly from surgical procedures. The patient is 81 years old and first experienced clotting due to hernia surgery in 1964, when he was hospitalized for 16 days post-surgery with life threatening complications. It was later determined in 1980, after many episodes, that the patient had hereditary protein C deficiency at the 38 % level. In his hernia surgery, perioperative ZPC was used along with accepted anticoagulation procedures with no blood clots or other related side effects occurring. This procedure can greatly benefit protein C deficient patients, and could potentially find use for non-PC deficient patients in surgeries and a variety of other medical treatments. This particular case helps to validate the importance of ZPC in effecting safer surgery in high-risk patients. It also supports the mechanism of ZPC acting as an anticoagulant without causing bleeding. Most importantly, each clinical case study represents a unique combination of surgeon, hematologist, medical staff, and patient functioning as a coordinated team. In this case, smaller amounts of very expensive ZPC achieved safe and efficacious results, which is hugely important for future clinical applications when considering the production cost of ZPC. More studies must be done to establish minimum dosing while achieving safe and efficacious outcomes.

Acquired hemophilia in patients with hematologic malignancies.

OBJECTIVES: To evaluate the occurrence of acquired hemophilia in patients with hematologic malignancies and to assess their response to treatment. DESIGN: Data on 8 patients with hematologic neoplastic disorders and inhibitor against factor VIII were analyzed retrospectively. SETTING: Three large tertiary-care centers. RESULTS: All 8 patients presented with spontaneous or posttraumatic hemorrhages. The mean inhibitor titer at the time of diagnosis was 79 Bethesda units (BU), and residual factor VIII activity was detectable in 3 patients. The inhibitor disappeared in 5 patients after a mean of 92 days, but persisted in the 3 other patients. The patients who achieved complete resolution of their circulating anticoagulant had lower mean inhibitor titers at the time of diagnosis than those who had persistent inhibitor (27 BU vs. 167 BU, respectively). Two patients died as a result of major hemorrhages that did not respond to treatment. CONCLUSIONS: Antibodies against factor VIII may be responsible for some bleeding episodes in patients with lymphoid or myeloid malignancies. Acquired hemophilia in this setting should be differentiated from other causes of bleeding because the approach to treatment is different. No conclusion can be drawn regarding the association between the activity of the underlying illness and the inhibitor titer, although it appears that at least in some patients such a relationship may exist. The underlying pathogenetic mechanisms responsible for the production of autoantibodies against factor VIII remain unclear, but we provide a few explanations in this article.

Genetic instability in patients with Hodgkin's disease undergoing chemotherapy.

We have studied the effect of chemotherapy on the level of a particular kind of genetic instability in patients with Hodgkin's disease. The particular type of genetic instability assayed is exemplified by trans-rearrangements between two (rather than within one) T cell antigen receptor. 16 patients were studied during their course of treatment. Presentation samples were available for 13 of these patients; 9 of them showed an increase in the level of trans-rearrangements during their exposure to chemotherapeutic agents (P < 0.043). All patients for whom posttherapy samples were available (10 out of 16) showed a return to baseline levels of trans-rearrangements 1-5 mo after completion of therapy (P < 0.03). Thus, this assay appears to be a marker for the "destabilizing" effects of certain chemotherapeutic agents.