Annemarie E. Fogerty

Marked hyperferritinemia does not predict for HLH in the adult population.

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of uncontrolled immune activation that has gained increasing attention during the last decade. The diagnosis of HLH is based on a constellation of clinical and laboratory abnormalities, including elevated serum ferritin levels. In the pediatric population, marked hyperferritinemia is specific for HLH. To determine what conditions are associated with profoundly elevated ferritin in the adult population, we performed a retrospective analysis in a large academic health care system. We identified 113 patients with serum ferritin levels higher than 50,000 µg/L. The most frequently observed conditions included renal failure, hepatocellular injury, infections, and hematologic malignancies. Our results suggest that marked hyperferritinemia can be seen in a variety of conditions and is not specific for HLH in adults.

Abdominal contouring procedures increase activity of the coagulation cascade.

AbstractOne of the most serious complications in plastic surgery is a thromboembolic event. However, little physiologic evidence exists to support the observed hypercoagulable state seen in contouring procedures. Twenty-one consecutive patients were enrolled prospectively to assess thrombin generation, which measures activity of the coagulation cascade, at baseline, intraoperative, and 24 hours after surgery. Compared with preoperative values, total thrombin generation increased by a mean of 997 nM intraoperatively (1.3-fold, P < 0.004) and 1406 nM postoperatively (1.4-fold, P < 0.001) in 9 patients undergoing abdominoplasty without deep venous thrombosis (DVT) chemoprophylaxis. The mean thrombin generation did not significantly change during or after surgery in 12 patients who received heparin for DVT prophylaxis (P = 0.3). Thrombin generation was significantly less in patients receiving chemoprophylaxis compared with those who received no prophylaxis (P < 0.01). This suggests abdominal contouring procedures induce a significant increase in the activity of the coagulation cascade that can be prevented by DVT chemoprophylaxis.

Management of inherited thrombophilia in pregnancy.

Purpose of reviewThis review summarizes the currently available data concerning risk and management of venous thromboembolism in pregnant women with inherited thrombophilia. SummaryPregnancy is a hypercoagulable state, and inherited thrombophilia increases this risk further. Despite the risks, the actual incidence of venous thromboembolism remains low, and therefore, the widespread use of anticoagulants for pregnant women with inherited thrombophilia is not advised. Although randomized, placebo-controlled trials investigating the risks and benefits of anticoagulation have not been performed, there are data to support the use of low molecular weight heparin for high and intermediate-risk women. We will review these data and treatment recommendations, which are based on retrospective and case–control studies as well as expert opinion and consensus statements.

Challenges of Anticoagulation Therapy in Pregnancy

Opinion statementThrombotic complications in pregnancy represent a major cause of morbidity and mortality. Pregnancy is a primary hypercoagulable state due to enhanced production of clotting factors, a decrease in protein S activity, and inhibition of fibrinolysis. These physiologic changes will yield a collective rate of venous thromboembolism (VTE) of about 1–2 in 1000 pregnancies for the general obstetric population, which represents a five- to tenfold increased risk in pregnancy compared to age-matched non-pregnant peers. A select group of women, however, will carry a significantly higher rate of thrombosis due to primary thrombophilia, either inherited or acquired. This introduces a population of women who may benefit from prophylactic anticoagulation, either antepartum or postpartum. The coagulation changes that occur in preparation for the hemostatic challenges of delivery endure for several weeks postpartum. In fact, daily risk for pulmonary embolism (PE) is the highest postpartum. Use of anticoagulation in pregnancy introduces particular risk at the time of delivery, where bleeding and clotting risk collide. Altered metabolism rates of anticoagulants in pregnant women often necessitate closer monitoring than is required outside of pregnancy in order to ensure efficacy and safety. Heparin products are the mainstay of treating VTE in pregnancy, chiefly because they do not cross the placenta. In women with mechanical heart valves, the ideal anticoagulation regimen remains controversial as heparin use has shown inferior outcomes for preventing thromboembolic complications compared to warfarin, but warfarin carries risk for fetal embryopathy. Other populations where a heparin alternative is necessary include women with a history of heparin-associated thrombocytopenia (HIT) or other heparin intolerance. Further challenging the management of anticoagulation in pregnancy is the dearth of randomized clinical trials. The evidence governing treatment recommendations is largely based on expert guidelines, observational studies, or extrapolation from non-pregnant cohorts. A careful critique of a woman’s history, as well as the available data, is essential for optimal management of anticoagulation in pregnancy. Such decisions should involve a multidisciplinary team involving obstetrics, hematology, cardiology, and anesthesia.

Case 36-2017

A 30-year-old man presented with fatigue, rash, anemia, and thrombocytopenia. Three years earlier, after an automobile accident, abdominal-wall hematomas, anemia, thrombocytopenia, and hematuria had developed but spontaneously resolved. A diagnostic test was performed.

Treating Venous Thromboembolism in Pregnancy

This review summarizes the currently available data concerning risk, diagnosis, and management of venous thromboembolism (VTE) in pregnant women. While the actual incidence of VTE in pregnancy remains low, the hypercoagulable state and increased risk requires clinicians to be aware of the specific aspects of presentation, diagnosis, and treatment in this population. The authors outline the strategies, efficacy, and safety of the diagnostic approach, as well as the available data concerning treatment. Considerations in treatment include maternal and fetal bleeding risk, as well as management of labor and delivery. The optimal approach to treatment requires collaboration between obstetrics, hematology, and anesthesia.

Thrombocytopenia in Pregnancy: Mechanisms and Management

Thrombocytopenia is a common hematologic issue encountered by obstetricians and hematologists, detected in about 10% of all pregnancies. In the vast majority of cases, the thrombocytopenia will be attributed to gestational thrombocytopenia (GT), where the thrombocytopenia is mild, does not necessitate active management, and does not introduce maternal or fetal bleeding risk. Although GT is common, the specific mechanism responsible for it is not known with certainty, and therefore, differentiating it from other causes of thrombocytopenia can be challenging. Previously proposed explanations for GT suggest that a decrease in platelet count is universal in pregnancy, and women diagnosed with GT are simply those with a baseline platelet count on the lower end of normal range. This concept is challenged in this review, and a possible mechanism for GT is proposed. Additionally, a framework for approaching the diagnosis and management of thrombocytopenia in pregnancy is presented.

Bleeding and Thrombosis in a Cancer Patient

Patients with cancer have an increased risk of developing venous thromboembolism (VTE) that is well documented, due to a variety of factors including tumor-induced hypercoagulability that varies by tumor type, stasis and vascular compression by tumor, and therapies used to treat the underlying malignancy. Advances in cancer detection and treatment have improved survival, even if patients are not cured, so that cancer in many patients can be viewed as a chronic disease, allowing patients to live longer but also develop complications. In addition to VTE, these complications can include an increased bleeding risk related to both tumor and treatments. Managing the competing risks of thrombosis and bleeding can be difficult in any patient and even more so in the cancer patient. Data regarding specific management is not always available, as each patient can present a unique combination of bleeding and thrombotic risks, and treatment must often be tailored to accommodate these individual risks. In the cases below, we review common clinical situations and discuss management strategies, with data to support these strategies when available.

Abdominal Contouring Procedures Are Associated with a Physiologic Hypercoagulable State

Background: One of the most serious complications from plastic surgery is a thromboembolic event. The incidence of thromboembolism in truncal contouring procedures ranges from 1-9%. However, little physiologic evidence exists to support the observed hypercoagulable state. This is the first study to assess physiological markers of hypercoagulability, and one of few prospective studies addressing thromboembolic risk and prophylaxis in body contouring patients.