Endoscopic Cyanoacrylate Injection Treatment for Refractory High-risk Peptic Ulcer Bleeding by Conventional Endoscopic Therapy

Bleeding is a major and potentially life-threatening complication of peptic ulcer. Despite endoscopic hemostatic therapy advance, conventional endoscopic hemostatic modalities remain refractory for peptic ulcer bleeding with big size, fibrous base or in difficult-to-access anatomical locations. In this study, we attempted to evaluate the efficacy and safety of endoscopic cyanoacrylate injection treatment (ECIT) for refractory high-risk peptic ulcer bleeding by conventional endoscopic therapy. The patients with refractory high-risk peptic ulcer bleeding by conventional endoscopic therapy were carried out ECIT. The data were retrospectively collected. A total of 119 patients accepted ECIT. 74 patients (62.18%) obtained successful intravascular injection and perivascular injection was performed in 45 patients (37.82%). Immediate hemostatic rate for active bleeding achieved 90.91%. Rebleeding rate within 30 days was 12.07%. Overall successful hemostasis rate achieved 87.93%. Immediate hemostatic rate and overall successful hemostasis rate in intravascular injection patients were markedly superior over perivascular injection. Rebleeding rate in intravascular injection patients was markedly lower than that in perivascular injection patients. 11 patients complicated abdominal pain and no other complication occurred. In conclusion, ECIT, especial intravascular injection, was effective and safe, with high successful hemostasis rate for refractory high-risk peptic ulcer bleeding by conventional endoscopic therapy.

Gestational hemostasis: a natural model for hemostasis resuscitation of major periprocedural blood loss

Early goal-directed treatment is an evidence-based approach to guide hemostatic therapy during major periprocedural bleeding. If viscoelastic coagulation tests are not available, an algorithm, termed the pyramid of hemostatic interventions, can help manage severe bleeding. Pregnant women accumulate huge reserves of prothrombotic and antifibrinolytic hemostatic elements to avoid peripartum blood loss. We provide comparison of therapeutic hemostatic approaches and natural gestational process and identified remarkable analogy between early goal-directed management of bleeding and hemostatic adaptation of pregnant woman. Therefore, gestational hemostasis serves as a natural model for goal-directed hemostasis resuscitation and can foster understanding of hemostatic management of periprocedural bleeding.

Orihime Study: Real World Treatment Patterns and Clinical Outcomes of 338 Patients with Acquired Hemophilia a from a Japanese Health Claims Database

Introduction: Acquired hemophilia A (AHA) is a rare disorder characterized by severe, spontaneous bleeding caused by autoantibodies against factor (F)VIII (inhibitors). It is known that onset of AHA is triggered by malignancy, autoimmune disease, dermatological disease, and pregnancy/delivery. As the standard therapy, immunosuppressive therapy (IST) should be started immediately to eliminate inhibitors and hemostatic therapy is also necessary in case of bleeding. Many patients require prolonged bed rest because of the bleeding risk; therefore, it is difficult to determine the best time to start rehabilitation. Additionally, the early deaths and high thrombotic rates are frequently reported in AHA. Since it is a rare disorder, the actual situation has not been fully clarified. This study was to describe the epidemiology and clinical practice of AHA in the real world using a large health claims database in Japan. Methods: This was a retrospective observational study using a health claims database provided by Medical Data Vision Co., Ltd. The data period was Apr. 2008-Mar. 2020. Patients who met all of the following criteria were included; patients with disease diagnosis of AHA; patients were hospitalized on the day of AHA diagnosis; and patients had immunosuppressants on/after the date of the first hospitalization. The first date of hospitalization was set as an Index date. Patients with disease diagnosis code of antiphospholipid syndrome, lupus anticoagulant, acquired factor XIII deficiency, acquired von Willebrand disease, or acquired factor V deficiency were excluded. Treatment/procedure patterns (IST, hemostatic therapy, and rehabilitation) and clinical outcome (Activities of Daily Living [ADL], death, and thromboembolism in the hospitalization) in AHA patients were investigated. Results: The study population of 338 patients (214 males: 124 females) was with the mean age of 75.7 (21-96) years. A total of 105 patients (pts) (31.1%) had concurrent diseases, including malignancy (61 pts, 18.0%), autoimmune diseases (40 pts, 11.8%), and dermatological diseases (18 pts, 5.3%). In bypassing agent use (153 pts, 45.3%), recombinant activated factor VII (rFVIIa) was the most frequently used (129 pts, 38.2%) followed by activated prothrombin complex concentrate (aPCC) (36 pts, 10.7%), and plasma-derived factor VIIa and factor X (FVIIa/FX) (14 pts, 4.1%). FVIII agent uses (8 pts) were very few. Median duration of treatment for bypassing agents ranged from 2.5 (FVIIa/FX) to 6.0 (aPCC) days. Steroids alone were used predominantly in the first line for immunosuppression (292 pts, 86.4 %) and oral prednisolone was the most frequently used. The category of rehabilitation most commonly implemented in AHA patients was disuse syndrome (104 pts, 30.8%) followed by locomotor (73 pts, 21.6%) and cerebrovascular (49 pts, 14.5%). Median time (days) from Index date to initiating rehabilitation was 16.5 for disuse syndrome, 23.0 for locomotor, 19.0 for cerebrovascular. In the total ADL scores (Barthel Index) in 196 patients with all 10 items, the proportions of patients with less than 70 points were high at both initial admission and final discharge (47.4% and 38.8%, respectively). The median number of times and length of hospitalization were 1.0 time and 62.0 days, respectively. Of evaluable population (328 pts), thromboembolism during hospitalization was recorded in 15 patients, by type of which disseminated intravascular coagulation (10 pts, 3.0%) was the most frequently recorded. Acute coronary syndrome (3 pts, 0.9%), pulmonary embolism and other (1 pt, 0.3%, each) were fewly recorded. The proportion of deaths during hospitalization was 18.6% (63 pts). Table 1 shows study result summary. Conclusions: This was the first study in a large AHA population using a health claims database in Japan. From an epidemiological point of view, the number of male patients was slightly larger and the mean age was slightly higher compared to the demographics in previous reports. The possible reason is regional variance or data source, whereas, the treatment patterns and the proportion of deaths during hospitalization are mostly aligned with the previous studies. Also this was the first report publishing the data on ADL and rehabilitation in AHA patients. The results showed that it took median 2-3 weeks to start rehabilitation. Further development of treatment strategies to enable early start of rehabilitation is awaited. Figure 1 Figure 1. Disclosures Ogawa: Chugai Pharmaceutical Co., Ltd.: Consultancy. Amano: Chugai Pharmaceutical Co., Ltd.: Consultancy, Speakers Bureau; KM Biologics Co., Ltd.: Research Funding, Speakers Bureau; Bioverativ Inc.: Speakers Bureau; Bayer AG: Speakers Bureau; Shire Plc: Speakers Bureau; Takeda Pharmaceutical Co., Ltd.: Speakers Bureau; Sanofi S.A.: Speakers Bureau; Novo Nordisk A/S: Speakers Bureau; CSL Behring: Speakers Bureau; Pfizer Inc.: Speakers Bureau; Japan Blood Products Organization: Speakers Bureau. Matsuo-Tezuka: Chugai Pharmaceutical Co., Ltd.: Current Employment. Okada: Chugai Pharmaceutical Co., Ltd.: Current Employment. Murakami: Chugai Pharmaceutical Co., Ltd.: Current Employment. Nakamura: Chugai Pharmaceutical Co., Ltd.: Current Employment. Yamaguchi-Suita: Chugai Pharmaceutical Co., Ltd: Current Employment. Nogami: Chugai Pharmaceutical Co., Ltd.: Consultancy, Honoraria, Research Funding, Speakers Bureau; Takeda Pharmaceutical Co., Ltd.: Honoraria, Research Funding, Speakers Bureau; CSL Behring: Honoraria, Research Funding, Speakers Bureau; Novo Nordisk A/S: Honoraria, Research Funding, Speakers Bureau; Bayer AG: Honoraria, Research Funding, Speakers Bureau; Sanofi S.A.: Honoraria, Research Funding, Speakers Bureau; KM Biologics Co., Ltd.: Honoraria, Research Funding, Speakers Bureau. OffLabel Disclosure: Data on the uses of cyclophosphamide, cyclosporin A, and rituximab for acquired hemophilia may be included in the poster presentation. However, with regard to these drugs, treatments for other diseases may also be included because this study was conducted with a secondary use of a health claims database. Off-label drug use is explained in the poster.

Transperitoneal hernioplasty in a patient with severe hemophilia A on preventive treatment with emicizumab

Performance of surgical interventions in patients with severe hemophilia A on emicizumab requires the development of a protocol for the perioperative period management. Objective. To present the first experience of laparoscopic hernioplasty, hemostatic therapy and laboratory monitoring in a patient with severe hemophilia A on emicizumab. A transperitoneal hernioplasty was performed in a 31-year-old patient with severe hemophilia A on emicizumab. The patient received hemostatic therapy with recombinant FVIII for 5 days. Laboratory parameters (detection of FVIII via chromogenic and clotting methods, thromboelastography, determination of aPTT and FVII inhibitor titer) were monitored for 8 days. For a complete postoperative hemostasis, a significantly smaller amount of FVIII concentrate was required due to the lower frequency of administrations compared to similar surgical interventions in patients with severe hemophilia A who did not receive prophylactic therapy with emicizumab. According to thromboelastrography data, not a single episode of hypercoagulation was recorded. Emicizumab monotherapy can maintain adequate hemostasis during surgical procedures associated with a potentially low risk of perioperative bleeding in patients with hemophilia A. In other situations, the use of standard doses of FVIII concentrate concomitantly with emicizumab makes it possible to control hemostasis during postoperative period without the risk of thrombotic complications. The patient has signed a consent to the use of information, including photos, for research purposes and in publications.

Tinjauan Terkini Hemofilia A yang Didapat : Aspek Diagnosis dan Manajemen

<p>Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.</p><p>Acquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.</p>