A 54-year-old male with biopsy-confirmed Gleason 4+4 prostate cancer underwent 18F-DCFPyL-PSMA PET scan to identify occult metastatic disease. This scan revealed abnormal radionuclide uptake not only in the prostate but also within the patient\'s vasculature. The scan was repeated after a week with a separate tracer batch, yielding the same result. Standard staging was performed using computed tomography and a Technetium-99 bone scan, revealing no metastatic disease. The patient\'s protein S deficiency is thought to have caused this peculiar tracer distribution. With the advent of PSMA PET for staging in prostate cancer, clinicians must be familiar with situations that may render unusual results.

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BACKGROUND: Protein S deficiency resulting in mesenteric vein thrombosis has been reported in previous studies however those causing SMA thrombosis has been rarely reported. Multidisciplinary approach involving general surgeon, a vascular surgeon, an interventional radiologist, and an intensivist are crucial for management of SMA thrombosis.
METHODS: A 39-year-old non-smoker hypertensive female who was diagnosed with partially occlusive thrombus in the superior mesenteric artery via Contrast-enhanced computed tomography (CECT) re-presented after 5 days and CECT revealed a partially occlusive thrombus in the superior mesenteric artery and Protein S deficiency (free protein S:15 %). She was managed by lysis of thrombus with streptokinase by interventional radiology team. The patient is on anticoagulants and without abdominal complaints on follow-up at 24 months.
CONCLUSIONS: Computed tomography angiography should be done immediately in any patient suspected of AMI since delay in diagnosis accounts for high mortality rates of 30-70 %. The surgical treatment of the condition is well established and consists of revascularization and/or resection of nonviable bowel. Endovascular techniques have emerged as an alternative for occlusion of the SMA. Patients with protein C and/or S deficiency treated for AMI require lifelong anticoagulant/antiplatelet therapy to prevent relapse.
CONCLUSIONS: Hereditary thrombophilia should be suspected in young people with unusual thrombotic presentations. Earlier diagnosis and aggressive antithrombotic therapy in individuals with hypercoagulable states can improve outcomes. Treatment involving a multidisciplinary approach improves outcomes.

Idiopathic purpura fulminans (IPF) is a rare and severe form of purpura fulminans caused by acquired protein S deficiency. It can lead to severe thrombotic complications, such as large skin necrosis and amputation. The lesions almost exclusively affect the lower limbs, and their distribution is similar among patients with IPF, unlike classical purpura fulminans lesions. Our hypothesis is that vascular structures called perforasomes may be involved in IPF, possibly caused by protein S deficiency. We analyzed all case reports and case series published in the literature that provided sufficient data for an anatomical study of limb injuries. For precise localization of areas of necrosis, we examined each case using descriptions and images to determine whether they overlapped with vascular territories that include perforasomes. We analyzed twelve cases from the literature and identified six vascular territories: the anterolateral, anteromedial, and posterior territories of the upper leg, as well as the anterolateral, anteromedial, and posterolateral territories of the lower leg. For each territory, we described the most probable vascular damage and the corresponding perforasome. IPF is a complex multifactorial disease in which a direct involvement of perforating arteries may be suspected and taken into account in the surgical of lesions.

Hereditary protein S (PS) deficiency is a rare condition associated with increased risk of venous thromboembolism (VTE). In 2020, the coronavirus disease 2019 (COVID-19) pandemic prompted development of vaccinations to protect against the virus. PS deficiency is not a contraindication to COVID-19 vaccinations, but there are no studies regarding potential adverse effects in this population. We report two cases, a 43-year-old mother and her 18-year-old son, who developed VTE shortly after their first COVID-19 vaccines. Testing confirmed hereditary PS deficiency with a previously undescribed mutation in both cases. The temporal association between COVID-19 vaccination and VTE in these patients with hereditary PS deficiency suggests a potential causal relationship. However, it is unclear if this applies to all patients with hereditary PS deficiency. This highlights the importance of reporting adverse events following COVID-19 vaccinations in this population to evaluate the risks and benefits of vaccination.

OBJECTIVE: To investigate the risk factors and underlying causes of pregnancy-related cerebral venous thrombosis (PCVT).
METHODS: A retrospective cohort of 16 patients diagnosed with CVT during pregnancy and postpartum (within six weeks after delivery) in a comprehensive hospital in China between 2009 and 2022 were carefully reviewed, focusing on demographic, clinical, and etiological characteristics, especially underlying causes. We matched 16 PCVT patients with 64 pregnant and puerperal women without PCVT to explore risk factors and clinical susceptibility to PCVT.
RESULTS: PCVT occurred commonly during the first trimester (43.75%) and the puerperium (37.5%). The frequency of anemia, thrombocytosis and thrombocytopenia during pregnancy, dehydration, and pre-pregnancy anemia was significantly higher in women with PCVT than in those without PCVT (P < 0.05). Among the 16 patients, five were diagnosed with antiphospholipid syndrome and one was diagnosed with systemic lupus erythematosus. Three patients had distinct protein S deficiency and one had protein C deficiency. Whole Exome Sequencing (WES) was performed for five patients and revealed likely pathogenic mutations associated with CVT, including heterozygous PROC c.1218G > A (p. Met406Ile), heterozygous PROS1 c.301C > T (p. Arg101Cys), composite heterozygous mutation in the F8 gene (c.144-1259C > T; c.6724G > A (p. Val2242Met)) and homozygous MTHFR c.677C > T (p. Ala222Val).
CONCLUSIONS: The occurrence of anemia, thrombocytopenia and thrombocytosis during pregnancy, dehydration and pre-pregnancy anemia suggested a greater susceptibility to PCVT. For confirmed PCVT patients, autoimmune diseases, hereditary thrombophilia, and hematological disorders were common causes. Screening for potential etiologies should be paid more attention, as it has implications for treatment and long-term management.

The mortality rate of gastric varices bleeding can reach 20% within 6 weeks. Isolated gastric varices (IGVs) refer to gastric varices without esophageal varices and typically arise as a common complication of left portal hypertension. Although IGVs commonly form in the setting of splenic vein occlusion, the combination of antiphospholipid syndrome and protein S deficiency leading to splenic vein occlusion is rare. We herein present a case of a 28-year-old woman with intermittent epigastric pain and melena. She was diagnosed with antiphospholipid syndrome based on the triad of pregnancy morbidity, unexplained venous occlusion, and positive lupus anticoagulant. Laparoscopic splenectomy and pericardial devascularization were performed for the treatment of IGVs. During the 6-month postoperative follow-up, repeated endoscopy and contrast-enhanced computed tomography revealed disappearance of the IGVs. This is the first description of splenic vein occlusion associated with both antiphospholipid syndrome and protein S deficiency. We also provide a review of the etiology, clinical manifestations, diagnosis, and treatment methods of IGVs.

Autoimmune diseases and thrombophilic disorders, notably antiphospholipid syndrome (APS) and protein S deficiency, present a formidable challenge in pregnancy, substantially increasing the risk of thromboembolic complications by up to 20%. Pulmonary thromboembolism (PTE), characterized by a significantly higher maternal mortality rate, is of particular concern. APS, defined by the presence of antiphospholipid antibodies, emerges as a pivotal risk factor for PTE during pregnancy, especially in women exhibiting triple negativity. Concurrently, protein S deficiency further amplifies vulnerability to thromboembolic events, establishing a high-risk scenario for pregnant individuals. In a case involving a 29-year-old pregnant woman with a history of generalized lupus erythematosus, triple-negative antiphospholipid syndrome, and protein S deficiency, sudden-onset dyspnea prompted thorough investigation. Despite her complex medical history, a multidisciplinary approach led to the accurate diagnosis and successful management of subsegmental pulmonary thromboembolism, ensuring the well-being of both mother and fetus. Effectively managing PTE during pregnancy demands a comprehensive, multidisciplinary approach involving collaboration among obstetricians, internists, rheumatologists, and hematologists. Accurate diagnosis, tailored anticoagulation strategies, and continuous monitoring stand as indispensable pillars for maternal and fetal well-being.

While direct oral anticoagulants (DOACs) are frequently used to treat venous thromboembolism (VTE), the outcomes of patients with inherited thrombophilia (IT) receiving DOACs for VTE remain understudied. We used data from the international RIETE registry to compare the rates of VTE recurrences, major bleeding, and mortality during anticoagulant treatment in VTE patients with and without IT, grouped by the use of DOACs or standard anticoagulant therapy. Among 103,818 enrolled patients, 21,089 (20.3%) were tested for IT, of whom 8422 (39.9%) tested positive: Protein C deficiency 294, Protein S deficiency 726, Antithrombin deficiency 240, Factor V Leiden 2248, Prothrombin gene mutation 1434, combined IT 3480. Overall, 14,189 RIETE patients (6.2% with IT) received DOACs, and 89,629 standard anticoagulation (8.4% with IT), mostly with heparins followed by vitamin K antagonists. Proportions of patients receiving DOACs did not differ between IT-positive and IT-negative patients. Rates of VTE recurrence on anticoagulant treatment were highest in patients with AT deficiency (P < 0.01). Rates of on-treatment major bleeding and all-cause mortality were lowest among patients with Factor V Leiden (FVL) or PT G20210A mutations, compared with patients who tested negative. Patients with IT who received DOACs had lower rates of major bleeding than those receiving standard anticoagulation. Excluding FVL and Protein S deficiency, patients with IT had lower rates of VTE recurrence with DOACs than with standard anticoagulation. DOACs are equally safe and effective in VTE patients with IT, with lower bleeding rates than those on standard anticoagulation.

Proteins C and S are vitamin K-dependent anticoagulative factors that also exert a significant influence on bone quality. Clinical studies have linked the deficiency of proteins C and S to lower bone mineral density and the onset of femoral head osteonecrosis in children. Rare foundational studies analyzing this topic have demonstrated that activated protein C, upon binding to the endothelial protein C receptor expressed on the surface of osteoblasts, promotes osteoblast proliferation. It is also established that proteins C and S play crucial roles in proper collagen synthesis and in maintaining the number of osteoclasts and blood vessels. However, the association between protein C and/or S deficiency and the gradual onset of osteoporosis remains largely uninvestigated. Calculations based on data from peer-reviewed journals suggest that approximately one in every 10 individuals may develop osteoporosis due to congenital protein C or S deficiency. Moreover, when secondary causes of protein C and S deficiency are also considered, the proportion likely further increases. In this paper, we discuss the pathophysiological background of the potential relationship between protein C and S deficiency and the genesis of osteoporosis.