Chakri Gavva

Transfusion management of factor V deficiency: three case reports and review of the literature

Factor V (FV) deficiency may be inherited as an autosomal recessive disease or acquired as a result of autoantibody formation, either spontaneously or secondary to exposure to bovine thrombin or medications. Congenital FV deficiency has traditionally been treated with plasma transfusions. However, recent evidence has suggested that platelet (PLT) transfusions may be a better alternative as FV stored within PLT alpha granules has greater procoagulant potential and is released locally at sites of vascular injury. We report three cases of FV deficiency, one congenital and two acquired, and emphasize the different management approaches.

Analysis of Thrombophilia Test Ordering Practices at an Academic Center: A Proposal for Appropriate Testing to Reduce Harm and Cost

Ideally, thrombophilia testing should be tailored to the type of thrombotic event without the influence of anticoagulation therapy or acute phase effects which can give false positive results that may result in long term anticoagulation. However, thrombophilia testing is often performed routinely in unselected patients. We analyzed all consecutive thrombophilia testing orders during the months of October and November 2009 at an academic teaching institution. Information was extracted from electronic medical records for the following: indication, timing, comprehensiveness of tests, anticoagulation therapy at the time of testing, and confirmatory repeat testing, if any. Based on the findings of this analysis, we established local guidelines in May 2013 for appropriate thrombophilia testing, primarily to prevent testing during the acute thrombotic event or while the patient is on anticoagulation. We then evaluated ordering practices 22 months after guideline implementation. One hundred seventy-three patients were included in the study. Only 34% (58/173) had appropriate indications (unprovoked venous or arterial thrombosis or pregnancy losses). 51% (61/119) with an index clinical event were tested within one week of the event. Although 46% (79/173) were found to have abnormal results, only 46% of these had the abnormal tests repeated for confirmation with 54% potentially carrying a wrong diagnosis with long term anticoagulation. Twenty-two months after guideline implementation, there was an 84% reduction in ordered tests. Thus, this study revealed that a significant proportion of thrombophilia testing was inappropriately performed. We implemented local guidelines for thrombophilia testing for clinicians, resulting in a reduction in healthcare costs and improved patient care.

Therapeutic plasma exchange for hypertriglyceridemia induced pancreatitis: A rapid and practical approach.

OBJECTIVES Acute hypertriglyceridemia induced pancreatitis (HTP) presents with a more severe clinical course compared to other etiologies of pancreatitis. Therapeutic plasma exchange (TPE) is a potential treatment option for lowering plasma triglycerides and possibly decreasing morbidity and mortality. However, clinical data regarding its effectiveness are limited. METHODS We retrospectively examined the clinical data and outcomes of 13 consecutive episodes of HTP in which TPE was employed to reduce plasma triglycerides during a 15-month period. RESULTS The TPE was initiated at a median of 19 hours from the time of presentation. We performed 1.2-1.5 volume TPEs with 5% albumin as the replacement fluid. After only one TPE procedure, the mean plasma triglycerides values decreased from 2993 mg/dl to 487 mg/dl with a reduction of 84%. All 13 patients survived with a mean length of hospital stay of 9.5 days. There were no complications related to TPE. CONCLUSIONS One TPE procedure is an effective method for reducing plasma triglycerides and possibly decreases the length of hospital stay in patients admitted with HTP.

How do we transfuse blood components in cirrhotic patients undergoing gastrointestinal procedures

The liver plays a pivotal role in hemostasis. Consequently, patients with cirrhosis frequently demonstrate abnormal coagulation profiles on routine laboratory tests. These tests mainly reflect decreased procoagulant proteins. However, in cirrhosis, complex changes also occur in anticoagulant and fibrinolytic pathways. Recent evidence demonstrates that patients with cirrhosis exist in a state of hemostatic rebalance. Accordingly, routine tests inadequately represent hemostatic alterations in these patients. Unfortunately, these tests are regularly used to guide the transfusion of blood components with the assumption that they will correct laboratory abnormalities and improve hemostasis in a bleeding patient or prevent excessive bleeding following a procedure. With an absence of both accurate laboratory testing to assess hemostasis and evidence‐based guidelines to direct the transfusion of blood components, management of patients with cirrhosis poses a significant challenge to clinicians. Therefore, we developed multidisciplinary guidelines for the periprocedural transfusion of blood components in patients with cirrhosis based on concurrent evidence and personal experience at our medical center.

Ischemic stroke in a patient with moderate to severe inherited factor VII deficiency

Thrombosis is known to occur in patients with rare inherited bleeding disorders, usually in the presence of a thrombotic risk factor such as surgery and/or factor replacement therapy, but sometimes spontaneously. We present the case of a 72-year-old African American male diagnosed with congenital factor VII (FVII) deficiency after presenting with ischemic stroke, presumably embolic, in the setting of atherosclerotic carotid artery stenosis. The patient had an international normalized ratio (INR) of 2.0 at presentation, with FVII activity of 6% and normal Extem clotting time in rotational thromboelastometry. He was treated with aspirin (325 mg daily) and clopidogrel (75 mg daily) with no additional bleeding or thrombotic complications throughout his admission. This case provides further evidence that moderate to severe FVII deficiency does not protect against thrombosis.

A case of autoimmune severe acquired von Willebrand syndrome (type 3-like)

Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency. We report a case of severe autoimmune AVWS in a woman with SLE who presented with clinical and laboratory features of type 3 VWD (undetectable VWF antigen, ristocetin cofactor activity, and VWF multimers). A mixing study demonstrated an inhibitor to VWF (6BU/mL). Her bleeds were managed with antifibrinolytics, recombinant activated factor VII, and activated prothrombin complex concentrate. She was initially treated with steroids and intravenous immunoglobulin therapy. However, her bleeding symptoms continued until she was treated with rituximab, and her VWF parameters normalized. She relapsed two years later due to non-compliance with her immunosuppressive medications and expired another two years later secondary to complications of sepsis and uremic pericarditis. This case emphasizes the importance of aggressive initial therapy of SLE to reduce secondary complications, frequent patient monitoring, and continued treatment of the underlying autoimmune disorder in patients with AVWS.

Four-factor prothrombin complex concentrates: effectiveness in the reversal of anticoagulation

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An Audit of Thrombophilia Testing in Patients with Ischemic Stroke or Transient Ischemic Attack: The Futility of Testing

OBJECTIVES Many patients admitted with an ischemic stroke or transient ischemic attack (TIA) undergo thrombophilia testing. There is limited evidence to support this practice. We examined the effect of thrombophilia testing on management of patients admitted with an ischemic stroke or TIA. MATERIALS AND METHODS In this retrospective observational single-center study, we identified patients who were admitted with stroke or TIA and underwent thrombophilia testing over a 45-month period. We reviewed their electronic medical records to assess whether testing affected clinical management, defined as anticoagulation treatment by the time of discharge due to a positive test result. Secondary endpoints included potential misdiagnosis due to false positive results and cost of testing. RESULTS Testing was performed in 143 patients with a stroke or TIA. Forty-four patients (31%) had at least 1 positive test result. The most common positive tests were an elevated factor VIII activity (18% of patients tested) and decreased protein S activity (11% of patients tested). Both of these tests are subject to acute phase effects. Testing altered clinical management in only 1 patient (1% of total patients tested). Thirty-three patients (75%) have the potential for carrying a misdiagnosis due to a positive test that was never repeated for confirmation or repeated too soon after the initial positive test. The annual cost of testing was approximately

A clinical audit of thrombophilia testing in pediatric patients with acute thromboembolic events: impact on management

62,000. CONCLUSIONS Thrombophilia testing in the acute inpatient setting rarely impacted the clinical management of patients admitted with a stroke or TIA. By avoiding thrombophilia testing, both the potential for misdiagnosis and health care costs can be reduced. Therefore, we have discontinued thrombophilia testing in in-patients with a diagnosis of stroke.

Response to Trans-2016-0405.R1

Routine testing for inherited and acquired thrombophilia defects is frequently performed in pediatric patients with thromboembolic events (TEEs). No consensus guidelines exist regarding the timing of testing or the type of patients to be tested. The primary objective of our study, therefore, was to determine whether thrombophilia testing during the acute TEE setting affected clinical management in pediatric patients. A secondary aim included estimation of potential harm from thrombophilia testing. We retrospectively reviewed data on all pediatric patients diagnosed with a TEE during a 1-year period. Fifty-two (51%) of 102 patients with a TEE underwent thrombophilia testing during the acute phase, with 26 patients (50%) having a positive test result during the acute phase. Only 12% of patients tested were confirmed to have a thrombophilia eventually, yielding a false-positive rate of ∼7% for testing when performed in the acute setting. There were no changes to the acute management, regardless of a positive or negative result. Testing resulted in unnecessary blood loss in 12 patients younger than 1 year and acute testing cost approximately