Background: Venous thromboembolism (VTE) complicates ∼1.2 of every 1000 deliveries. Despite these low absolute risks, pregnancy-associated VTE is a leading cause of maternal morbidity and mortality.
...Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians and others in decisions about the prevention and management of pregnancy-associated VTE.
Methods: ASH formed a multidisciplinary guideline panel balanced to minimize potential bias from conflicts of interest. The McMaster University GRADE Centre supported the guideline development process, including updating or performing systematic evidence reviews. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess evidence and make recommendations.
Results: The panel agreed on 31 recommendations related to the treatment of VTE and superficial vein thrombosis, diagnosis of VTE, and thrombosis prophylaxis.
Conclusions: There was a strong recommendation for low-molecular-weight heparin (LWMH) over unfractionated heparin for acute VTE. Most recommendations were conditional, including those for either twice-per-day or once-per-day LMWH dosing for the treatment of acute VTE and initial outpatient therapy over hospital admission with low-risk acute VTE, as well as against routine anti-factor Xa (FXa) monitoring to guide dosing with LMWH for VTE treatment. There was a strong recommendation (low certainty in evidence) for antepartum anticoagulant prophylaxis with a history of unprovoked or hormonally associated VTE and a conditional recommendation against antepartum anticoagulant prophylaxis with prior VTE associated with a resolved nonhormonal provoking risk factor.
Background: Venous thromboembolism (VTE) is a common source of perioperative morbidity and mortality.
Objective: These evidence-based guidelines from the American Society of Hematology (ASH) intend ...to support decision making about preventing VTE in patients undergoing surgery.
Methods: ASH formed a multidisciplinary guideline panel balanced to minimize bias from conflicts of interest. The McMaster University GRADE Centre supported the guideline-development process, including performing systematic reviews. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess evidence and make recommendations, which were subject to public comment.
Results: The panel agreed on 30 recommendations, including for major surgery in general (n = 8), orthopedic surgery (n = 7), major general surgery (n = 3), major neurosurgical procedures (n = 2), urological surgery (n = 4), cardiac surgery and major vascular surgery (n = 2), major trauma (n = 2), and major gynecological surgery (n = 2).
Conclusions: For patients undergoing major surgery in general, the panel made conditional recommendations for mechanical prophylaxis over no prophylaxis, for pneumatic compression prophylaxis over graduated compression stockings, and against inferior vena cava filters. In patients undergoing total hip or total knee arthroplasty, conditional recommendations included using either aspirin or anticoagulants, as well as for a direct oral anticoagulant over low-molecular-weight heparin (LMWH). For major general surgery, the panel suggested pharmacological prophylaxis over no prophylaxis, using LMWH or unfractionated heparin. For major neurosurgery, transurethral resection of the prostate, or radical prostatectomy, the panel suggested against pharmacological prophylaxis. For major trauma surgery or major gynecological surgery, the panel suggested pharmacological prophylaxis over no prophylaxis.
Liver transplant has occasionally been performed in the presence of congenital afibrinogenemia and has been rarely used to treat it. Historically, to safely manage coagulopathy during transplant, ...these patients have been administered a combination of fresh frozen plasma and cryoprecipitate. In this case report, we discuss the first reported use of recombinant fibrinogen to treat such a patient and the decision-making process considered to balance the thrombotic and hemorrhagic risks.
Early and aggressive treatment of trauma-associated coagulopathy through transfusion of high plasma to packed red blood cell ratios is gaining favor. Whether this strategy is associated with improved ...survival is unclear. We performed a systematic review to determine whether higher plasma to packed red blood cell ratios compared with lower plasma to packed red blood cell ratios were associated with a survival advantage.
We searched electronic databases MEDLINE, Embase, and Web of Science from 1950 to February 2010 for studies comparing mortality in massively transfused trauma cohorts receiving different plasma to packed red blood cell ratios.
Two reviewers independently performed study selection. Discrepancies in study selection were resolved by discussion and consensus.
Two reviewers independently extracted data from each study using a standardized form. Two authors independently assessed study quality using the Newcastle-Ottawa Scale.
Eleven observational studies and no randomized controlled trials were identified. Three studies found a survival benefit with a 1:1 plasma to packed red blood cell transfusion ratio compared with either higher or lower ratios. Six studies did not examine a 1:1 ratio but concluded that higher plasma to packed red blood cell ratios improved survival. Secondary outcomes, including multiorgan system failure, packed red blood cell transfusion, respiratory outcomes, and coagulation variables, did not uniformly favor 1:1 or higher plasma to packed red blood cell ratios.
Methodological flaws, including survival bias, and heterogeneity between studies preclude statistical comparisons concerning the effects of a 1:1 plasma to packed red blood cell transfusion ratio. There is insufficient evidence to support a survival advantage with a 1:1 plasma to packed red blood cell transfusion strategy. Randomized controlled trials evaluating safety and efficacy are warranted before a high plasma to packed red blood cell transfusion ratio can be recommended.
Choosing Wisely® is a medical stewardship initiative led by the American Board of Internal Medicine Foundation in collaboration with professional medical societies in the United States. The American ...Society of Hematology (ASH) released its first Choosing Wisely® list in 2013. Using the same evidence-based methodology as in 2013, ASH has identified 5 additional tests and treatments that should be questioned by clinicians and patients under specific, indicated circumstances. The ASH 2014 Choosing Wisely® recommendations include: (1) do not anticoagulate for more than 3 months in patients experiencing a first venous thromboembolic event in the setting of major, transient risk factors for venous thromboembolism; (2) do not routinely transfuse for chronic anemia or uncomplicated pain crises in patients with sickle cell disease; (3) do not perform baseline or surveillance computed tomography scans in patients with asymptomatic, early-stage chronic lymphocytic leukemia; (4) do not test or treat for heparin-induced thrombocytopenia if the clinical pretest probability of heparin-induced thrombocytopenia is low; and (5) do not treat patients with immune thrombocytopenia unless they are bleeding or have very low platelet counts.
Objectives
This study aimed to assess the effectiveness of a continuous quality improvement initiative at the University of Florida Health Physicians practice in reducing the time to administer ...factor replacement therapy (FRT) for hemophilia patients presenting with bleeding in the emergency department (ED).
Methods
The study, a quasi‐experimental, interventional design, was conducted between January 2020 and January 2023. The intervention, implemented in September 2021, involved training ED physicians, creating a specialized medication order set within the electronic health record (EHR), and a rapid triage system. The effectiveness was measured by comparing the time from ED arrival to factor administration before and after the intervention and benchmarking it against the National Bleeding Disorders Foundation's Medical and Scientific Advisory Council (MASAC)‐recommended 1‐hour timeline for factor administration. An interrupted time series (ITS) analysis with a generalized least squares model assessed the intervention's impact.
Results
A total of 43 ED visits (22 pre‐intervention and 21 post‐intervention) were recorded. Post‐intervention, the average time from ED arrival to factor administration decreased from 5.63 to 3.15 hours. There was no significant increase (27% vs. 29%) in the patients receiving factor within 1‐hour of ED arrival. The ITS analysis predicted a 20‐hour reduction in the average quarterly time to administer factor by the end of the study, an 84% decrease.
Conclusions
The quality improvement program decreased the time to administer FRT for patients with hemophilia in the ED. However, the majority of patients did not achieve the 1‐hour MASAC‐recommended timeline for factor administration after ED arrival.
Introduction: Thrombotic Thrombocytopenic Purpura (TTP) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and is often associated with ...renal failure and neurologic manifestations. There are congenital and acquired forms of TTP both diagnosed by severe deficiency in ADAMTS13 a von Willebrand factor cleaving protease. Atypical Hemolytic Uremic Syndrome (aHUS), is also a rare disease with MAHA, thrombocytopenia, and acute kidney injury not related with Escherichia coli 0157:H7 infections and is caused by unregulated activation of the alternate complement pathway.
Although established that pregnancy may predispose to TTP and aHUS, little is understood regarding outcomes and effect on subsequent pregnancies. We report clinical outcomes of patients with TTP and aHUS during pregnancy at University of Florida (UFH).
Methods: Pregnant patients between age 18- 45 admitted to UFH between January 2010 - December 2016 with clinical diagnosis of TMA (TTP, atypical HUS, HUS, drug induced HUS,) were identified by ICD 9/10 codes (ICD 10- CM - M31.1, ICD-9-CM 446, ICD 10 - D59.3 , ICD 09 - 283.11, Z34 and V22, O09 and V23). A total of 55 patients were identified of which 2 patients had TTP and 1 had aHUS. Data including demographics, race, gestational age at diagnosis of TMA, timing of ADAMTS13 testing, presence of fever, diarrhea, neurological symptoms, petechiae, bleeding , laboratory data (complete blood count, complete metabolic profile, coagulation panel, LDH ) , peripheral smear results; timing of plasmapheresis(PLEX), use of rituximab/ eculizumab, length of stay, 30 day readmission rate, days required for platelet recovery, number of PLEX sessions conducted were obtained and documented on an excel sheet. Given that 3 patients met study criteria, descriptive analysis was performed.
Results: Of the 2 patients with TTP, patient 1 had 5 pregnancies and patient 2 had 1 pregnancy (fig 1). Altered mental status was the common feature in both patients (fig 3). Diagnosis of TTP was made on day 1 with confirmation of diagnosis between day 3-5. They were started on plasma infusion, steroids and plasmapheresis within 24 hrs. Response to treatment was variable with time to platelet recovery ranging between 3-10 days. Both patients remained in remission while not pregnant. Amongst the 6 pregnancies between them 1 resulted in Intrauterine Fetal Death, 2 elective abortions and 3 live births. Maternal survival was 100%. Patient 1 was eventually found to have ADAMTS 13 inhibitor and sarcoidosis and none of them had congenital TTP. Patient 3 presented with features of HELLP at 31 week of gestation and had emergent c- section after which she developed acute kidney injury, worsening thrombocytopenia and anemia. Was initially treated for TTP and started on PLEX, with minimal response after 5 sessions. ADAMTS 13 >35% and kidney biopsy showed TMA. She was diagnosed with aHUS and started on eculizumab, after which she showed clinical improvement. Subsequent follow up showed that there were no long-term health concerns in the children.
Discussion: The diagnosis of TTP and aHUS during pregnancy can be challenging, as this condition mimics several other diseases like HELLP and acute fatty liver of pregnancy. Owing to the devastating nature, it is imperative to make the right diagnosis in a timely manner. Hematologists are often also asked to estimate risk of recurrence of TTP during pregnancy and also impact on the pregnancy itself. Jiang et al using the Oklahoma (TTP-HUS) Registry showed that women with a previous history of TTP, the frequency of recurrence is low and pregnancy outcomes are positive. Our review showed favorable outcome of pregnancy in patients with TTP and aHUS when diagnosis is made in a timely manner and treated appropriately i.e, PLEX within 24 hours of diagnosis. Our study also demonstrates that in patients with aHUS, PLEX stabilized the patient, but complete response is achieved only with Eculizumab, a humanized recombinant monoclonal antibody that inhibits the terminal pathway of complement activation by blocking the activation of complement protein C5. Without eculizumab, the abnormal pattern of complement activation and TMA are likely to persist with the risk of irreversible organ damage. Our study has limitations owing to the small study population. TTP and aHUS in pregnancy are however very rare and we hope to add to the existing literature.
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Rajasekhar:Alexion: Membership on an entity's Board of Directors or advisory committees; Bayer: Membership on an entity's Board of Directors or advisory committees; Octapharma: Membership on an entity's Board of Directors or advisory committees; Kedrion Biopharma: Membership on an entity's Board of Directors or advisory committees; ABIM: Other: direct payment for serving on Hematology Board Question Writing Task Force.
Learning Objectives
After completing this course, the reader will be able to:
Describe the clinical and radiologic manifestations of RPLS.
Define agents or inciting risk factors that have been ...implicated in the development of RPLS.
Describe the treatment and outcome of RPLS, emphasizing supportive care and urgent discontinuation of the offending agent.
Access and take the CME test online and receive 1 AMA PRA Category 1 Credit™ at CME.TheOncologist.com
Gemcitabine is a commonly used chemotherapeutic agent for a variety of tumor types. Although this nucleoside analogue antineoplastic agent is similar in structure to cytarabine, central nervous system toxicities have rarely been attributed to gemcitabine. Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare but increasingly identifiable clinicoradiologic process in cancer patients associated with cytotoxic and immunosuppressive agents. The syndrome is characterized by acute to subacute onset of headache, nausea, vomiting, altered mental status, seizures, stupor, and visual disturbances. The pathophysiology of RPLS continues to remain controversial but likely involves loss of cerebrovascular autoregulation leading to arteriole leakage. Radiologically, posterior occipital white matter edema is noted, with characteristic findings on magnetic resonance imaging. Often the syndrome is reversible with treatment of concurrent hypertension or removal of the causative agent; however, failure to quickly recognize the syndrome and discontinue the offending agent may result in profound and permanent central nervous system dysfunction or death. This article describes a case of RPLS attributed to gemcitabine use for pancreatic cancer. Such a descriptive case serves as a platform for the discussion of the syndrome, proposed mechanisms of central nervous system damage, and review of the currently available literature on the topic. With increased awareness of RPLS by oncologists and other medical providers, cancer patient care may be improved and further insight into this complication of therapy through continued research may be gained.
Venous thromboembolism (VTE) is a common cause of inpatient and outpatient morbidity and mortality. While anticoagulant therapy is considered the primary means of prevention and treatment of VTE, ...inferior vena cava filters (IVCFs) are often used as an alternative or adjunct to anticoagulation. With the advent of retrievable filters indications have liberalized, to include placement for primary prophylaxis in high-risk patients. However, this practice is based on limited evidence supporting their efficacy in preventing clinically relevant outcomes. Since indiscriminate use of IVCFs can be associated with net patient harm and increased health care costs, knowledge of the literature surrounding IVCF utilization is critical for providers to adopt best practices. In this review, we will provide an overview of the literature as it relates to specific clinical questions that arise when considering IVCF utilization in the prevention and treatment of VTE. Practice-based recommendations will be reviewed to provide the clinician with guidance on challenging clinical scenarios.
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