A Case Report of Pulmonary Embolectomy Under Cardiopulmonary Bypass in a Patient with Intracranial Hemorrhage and History of Heparin-Induced Thrombocytopenia.

Pulmonary embolism is a common complication after intracranial hemorrhage. As thrombolysis is contraindicated in this situation, surgical pulmonary embolectomy may be indicated in case of high-risk pulmonary embolism but requires transient anticoagulation with heparin during cardiopulmonary bypass. We report the case of a patient with a history of heparin-induced thrombocytopenia who presented with a high-risk pulmonary embolism 10 days after the spontaneous onset of a voluminous intracerebral hematoma. Despite high doses of heparin required to run the cardiopulmonary bypass and subsequent anticoagulation by danaparoid sodium, the brain hematoma remained stable and the patient was discharged without complications 30 days after surgery.

Auxiliary liver xenotransplantation technique in a transgenic pig-to-non-human primate model: A surgical approach to prolong survival.

Xenotransplantation using pigs' liver offers a potentially alternative method to overcome worldwide donor shortage, or more importantly as a bridge to allotransplantation. However, it has been challenged by profound thrombocytopenia and fatal coagulopathy in non-human primate models. Here we suggest that a left auxiliary technique can be a useful method to achieve extended survival of the xenograft. Fifteen consecutive liver xenotransplants were carried out in a pig-to-cynomolgus model. Right auxiliary technique was implemented in two cases, orthotopic in eight cases, and left auxiliary in five cases. None of the right auxiliary recipients survived after surgery due to hemorrhage during complex dissection between the primate's right lobe and inferior vena cava. Orthotopic recipients survived less than 7 days secondary to profound thrombocytopenia and coagulopathy. Two out of five left auxiliary xenotransplants survived more than 3 weeks without uncontrolled thrombocytopenia or anemia, with one of them surviving 34 days, the longest graft survival reported to date. Left auxiliary xenotransplant is a feasible approach in non-human primate experiments, and the feared risk of thrombocytopenia and coagulopathy can be minimized. This may allow for longer evaluation of the xenograft and help better understand histopathological and immunological changes that occur following liver xenotransplantation.

Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia.

BACKGROUND AND PURPOSE: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. METHODS: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680-1689), were included. RESULTS: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. CONCLUSIONS: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.

Characteristics of Snakebite-Related Infection in French Guiana.

Wound infection is frequently reported following snakebite (SB). This study is retrospective. It was conducted in the emergency department and the Intensive Care Unit (ICU) of Cayenne General Hospital between 1 January 2016 and 31 July 2021. We included 172 consecutive patients hospitalized for SB envenoming. All patients were monitored for wound infection. Sixty-three patients received antibiotics at admission (36.6%). The main antibiotic used was amoxicillin-clavulanate (92.1%). Wound infection was recorded in 55 cases (32%). It was 19% in grade 1, 35% in grade 2, and 53% in grade 3. It included abscess (69.1%), necrotizing fasciitis (16.4%), and cellulitis (21.8%). The time from SB to wound infection was 6 days (IQR: 3-8). The main isolated microorganisms were A. hydrophila and M. morganii (37.5% and 18.8% of isolated organisms). Surgery was required in 48 patients (28.1%), and a necrosectomy was performed on 16 of them (33.3%). The independent factors associated with snakebite-associated infection were necrosis (p < 0.001, OR 13.15, 95% CI: 4.04-42.84), thrombocytopenia (p = 0.002, OR: 3.37, 95% CI: 1.59-7.16), and rhabdomyolysis (p = 0.046, OR: 2.29, 95% CI: 1.02-5.19). In conclusion, wound infection following SB is frequent, mainly in grade 2 and 3 envenomed patients, especially those with necrosis, thrombocytopenia, and rhabdomyolysis. The main involved bacteria are A. hydrophila and M. morganii.

Sphenopalatine Ganglion Block for Postdural Puncture Headaches in a Thrombocytopenic Adolescent Young Adult: A Case Report.

The epidural blood patch is the gold-standard therapy to treat postdural puncture headaches from dural puncture when conservative therapies fail. However, an epidural blood patch is contraindicated in patients with coagulopathy and thrombocytopenia (platelet count of 60,000/µL) due to concerns of an epidural hematoma. We present a case of an adolescent with acute lymphocytic leukemia, thrombocytopenia, and persistent postdural puncture headaches. The patient was successfully treated with a sphenopalatine ganglion nerve block at the bedside without any side effects and complications.

Indium-labelled autologous platelet sequestration studies predict response to splenectomy in immune thrombocytopenia: an Australian experience.

BACKGROUND: Splenectomy is an effective intervention in primary immune thrombocytopenia (ITP). Attempts to define pre-clinical predictors of platelet response to splenectomy are inconsistent. Based on international studies defining the likelihood of platelet response using platelet sequestration, patients with relapsed/refractory ITP being considered for splenectomy at a regional Australian hospital were assessed with 111 indium-labelled autologous platelet sequestration (ILAPS) studies. AIMS: To audit the use of ILAPS in an Australian setting and define its role in predicting response to splenectomy. METHODS: A retrospective review of all patients referred for an ILAPS study at a regional hospital was performed. Results for each patient were expressed as an 'R' value (spleen/ liver uptake ratio) to quantify the platelet sequestration pattern and outcome post-splenectomy, based on platelet counts. RESULTS: A total of 45 patients was identified: 13 underwent splenectomy and 32 were medically managed. Patients with favourable ILAPS scans (pure or predominant splenic sequestration) demonstrated a superior response post-splenectomy (100% overall response rate (ORR); 83.5% complete remission (CR)) compared with those with unfavourable ILAPS scans (mixed or pure hepatic sequestration) (71.4% ORR; 57.1% CR) over 12 months. CONCLUSIONS: The use of ILAPS in the Australian setting is feasible and this experience confirms larger international studies demonstrating its utility as a predictor of response to splenectomy in ITP. An unfavourable ILAPS scan could be considered a negative predictor of response prompting consideration for other emerging ITP treatments such as thrombopoietin-receptor agonists or B-cell depleting therapy such as Rituximab.

Thrombocytopaenia successfully treated by a multilocular thymic cyst resection.

The multilocular thymic cyst (MTC) is a rare, acquired disease caused by inflammatory changes in the thymus, and is associated with autoimmune diseases. We report a case of MTC with thrombocytopaenia, which improved following surgical resection. A 45-year-old man developed thrombocytopaenia with an anterior mediastinal tumour. Thrombocytopaenia due to an autoimmune mechanism, associated with thymoma or thymus-related disease, was suspected. Pathologic analysis following thoracoscopic thymectomy confirmed MTC. The platelet level recovered postoperatively. Our findings suggested a relationship between the acquired formation of MTC and the development of autoimmune antibodies. However, further investigation is needed to obtain more information.

Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study.

OBJECTIVE: Evans syndrome, the combination of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) or autoimmune neutropenia, is associated with a high rate of relapsed/refractory disease. There are limited data on the efficacy of splenectomy for this condition. We reviewed patient outcomes after splenectomy for Evans syndrome compared to ITP at our institution. METHODS: We performed a retrospective analysis of patients who underwent splenectomy for autoimmune cytopenias over a 23-year period with the intention of comparing disease relapse rates after splenectomy in patients with Evans syndrome and in those with ITP. RESULTS: During the study period, 77 patients underwent splenectomy for ITP and seven underwent splenectomy for Evans syndrome. In the Evans cohort, splenectomy led to an 85.7% initial response rate with a 42.8% rate of relapse within one year and a long-term (one-year) response rate of 42.8%. In the ITP cohort, the initial response rate was 90.9% with a long-term response rate of 70.1%. CONCLUSION: Our data suggest that long-term remission rates after splenectomy are lower in adults with Evans syndrome compared to those with ITP, although splenectomy may still be an acceptable treatment for certain patients with Evans syndrome. Our findings underscore the need for further research and development of additional therapeutic strategies for this patient population.

Successful cardiac surgery in a patient with Evans syndrome.

Evans syndrome is a rare haematological disease that may cause several complications during heart surgery. Herein we documented heart valve surgery in a patient with Evans syndrome who was receiving monoclonal antibody therapy, and valve replacement was successfully performed via prophylactic measures against haemolysis.

Successful Treatment of an Adolescent Male With Severe Refractory Evans Syndrome Using Bortezomib-based Therapy.

Evans syndrome is defined by bilineal autoimmune cytopenia, typically coombs positive hemolytic anemia and thrombocytopenia. Corticosteroids are the mainstay of treatment, with rituximab and/or mycophenolate mofetil often used in steroid-refractory cases. However, no treatment methodology has ever evaluated by a randomized clinical trial. We present a 15-year-old boy with Evans syndrome and common variable immunodeficiency who experienced a severe, refractory flare 16 months postsplenectomy. After failing to respond to multiple other agents, he achieved a durable response to a bortezomib-based regimen. Bortezomib may be a reasonable second or third line option, especially before high-morbidity therapies such as splenectomy or stem cell transplantation.

Point-of-Care Thromboelastography for Intrathecal Drain Management in Patients With Coagulopathy and Thoracic Aorta Surgery: A Case Report.

Spinal drain placement to prevent spinal cord ischemia during thoracic aorta surgery is a necessary yet complex undertaking in patients with coagulopathies. Thromboelastography (TEG) can be used as a point-of-care management tool to monitor coagulation status before drain placement and removal. We present 2 cases: a case of a patient with factor VII deficiency and a case of a patient with thrombocytopenia for whom TEG was an important procedural adjunct during coagulopathy reversal. TEG parameters are also discussed to encourage more frequent TEG use as an adjunct during these complex cases.

Pharmacokinetic/Pharmacodynamic Modelling and Simulation of Lusutrombopag, a Novel Thrombopoietin Receptor Agonist, for the Treatment of Thrombocytopenia in Patients with Chronic Liver Disease Undergoing Invasive Procedures.

BACKGROUND: Patients with thrombocytopenia associated with chronic liver disease (CLD) are at greater risk of bleeding during invasive procedures. This study characterized the pharmacokinetic/pharmacodynamic (PK/PD) profile of lusutrombopag, a novel thrombopoietin-receptor agonist, using modelling and simulation, and evaluated the appropriate dose regimen for treatment of thrombocytopenia in CLD patients undergoing invasive procedures. METHODS: A population PK/PD model was developed using plasma lusutrombopag concentrations from 78 healthy subjects and 349 CLD patients, as well as platelet counts from 347 of these 349 patients. Covariates were explored from subject characteristics. Monte-Carlo simulations were performed to assess a dose response for efficacy (platelet counts ≥ 50,000/µL) and a risk for platelet overshooting (platelet counts > 200,000/µL). RESULTS: Visual predictive checks indicated the developed models described the PK/PD profile of lusutrombopag well. In the simulations, without stopping criteria, lusutrombopag 3 mg once daily for 7 days before scheduled invasive procedures provided effective platelet response (85.2% probability for efficacy). The probability of platelet overshooting was 1.2%, indicating that platelet monitoring is not necessary. Although body weight was an influential covariate on the pharmacokinetics of lusutrombopag, individually estimated peak platelet counts overlapped among the body weight groups, suggesting no clinically significant effect on body weight. CONCLUSION: The modelling and simulation support lusutrombopag 3 mg once daily for 7 days without platelet monitoring.

Thrombocytopenia and declines in platelet counts: predictors of mortality and outcome after mechanical thrombectomy.

BACKGROUND AND PURPOSE: Acute ischemic stroke (AIS) has well-known risk factors. The role of platelets in patients treated using mechanical thrombectomy (MT) has not been studied. The aim of this study was to study if there is an association of initial thrombocytopenia (TP) and a decline of platelets counts (DPC) with the clinical outcomes, mortality and intracranial hemorrhage (ICH) rates in AIS patients treated with MT. MATERIALS AND METHODS: In a case-control study consecutive MT-stroke patients were analyzed. A multivariate logistic regression model was used to test for good clinical outcome (mRS 90 days <= 2) and mortality adjusting for age, initial NIHSS, pretreatment with tPA, statins and platelet inhibitors, occlusion site, time from symptom onset to recanalization, initial TP (< 150 × 109/L) and DPC (> 26%). Additionally, rates of ICH were compared. RESULTS: Of 294 patients included, 9.6% had an initial TP and 23.8% a DPC > 26%. The mortality rate in patients with normal platelet counts was 26.1% vs. 48.3% (p = 0.002) in patients with initial TP with an aOR of 3.47 (CI 1.28-9.4, p = 0.005). No difference regarding the rate of good clinical outcome (p = 0.204) and ICH (p = 0.18) was observed. A DPC of more than 26% during the first 5 days of hospitalization predicted the rate of mortality (aOR 2.4 CI 1.14-5.04, p = 0.021) and the chances of a good clinical outcome (aOR 0.291 CI 0.128-0.666, p = 0.003) without significant differences of ICH rates (p = 0.735). CONCLUSION: In AIS patients treated with MT an initial TP was independently associated with higher mortality rates and a marked DPC with higher mortality rates as well as poorer clinical outcomes.

Preoperative Thrombocytopenia May Predict Poor Surgical Outcome after Extended Hepatectomy.

Background: It is a novel idea that platelet counts may be associated with postoperative outcome following liver surgery. This may help in planning an extended hepatectomy (EH), which is a surgical procedure with high morbidity and mortality. Aim: The aim of this study was to evaluate the predictive potential of platelet counts on the outcome of EH in patients without portal hypertension, splenomegaly, or cirrhosis. Methods: A series of 213 consecutive patients underwent EH (resection of ≥ five liver segments) between 2001 and 2016. The association of preoperative platelet counts with posthepatectomy liver failure (PHLF), morbidity (based on Clavien-Dindo classification), and 30-day mortality was evaluated using multivariate analysis. Results: PHLF was detected in 26.3% of patients, major complications in 26.8%, and 30-day mortality in 11.3% of patients. Multivariate analysis revealed that the preoperative platelet count is an independent predictor of PHLF (odds ratio [OR] 4.4, 95% confidence interval [CI] 1.3-15.0, p=0.020) and 30-day mortality (OR 4.4, 95% CI 1.1-18.8, p=0.043). Conclusions: Preoperative platelet count is associated with PHLF and mortality following extended liver resection. This association was independent of other related parameters. Prospective studies are needed to evaluate the predictive role and to determine the impact of preoperative correction of platelet count on postoperative outcomes after EH.

Indication of total parathyroidectomy for an Epstein syndrome patient with end-stage renal disease.

Epstein syndrome is an extremely rare genetic disorder characterized by the association of nephritis, deafness and megathrombocytopenia. We present the case of a 21-year-old patient diagnosed with Epstein syndrome and hyperparathyroidism secondary to chronic kidney disease. The main particularity of this case resides in the association between megathrombocytopenia and secondary hyperparathyroidism requiring surgery, which could lead to a series of concerns regarding the intra- and postoperative hemorrhagic risk of the procedure. Nevertheless, both the surgical procedure and the postoperative recovery were uneventful, suggesting that the lower threshold for preoperative thrombocyte count in megathrombocytopenia should be specifically considered on an individual case analysis.