• Antiphospholipid syndrome
• antiphospholipid antibodies

• Humans
• Female
• Thrombocytopenia/epidemiology
• Thrombocytopenia/immunology
• Thrombocytopenia/etiology
• Male
• Registries
• Antiphospholipid Syndrome/complications
• Antiphospholipid Syndrome/immunology
• Antiphospholipid Syndrome/blood
• Antiphospholipid Syndrome/drug therapy
• Retrospective Studies
• Middle Aged
• Antibodies, Antiphospholipid/blood
• Antibodies, Antiphospholipid/immunology
• Adult
• Anemia, Hemolytic, Autoimmune/epidemiology
• Anemia, Hemolytic, Autoimmune/immunology
• Anemia, Hemolytic, Autoimmune/etiology
• Lupus Erythematosus, Systemic/immunology
• Lupus Erythematosus, Systemic/complications
• Databases, Factual
• Immunosuppressive Agents/therapeutic use
• Aged

• Anticoagulants
• antiphospholipid syndrome (APS)
• antithrombotic therapy
• bleeding
• thrombocytopenia

• Humans
• Antiphospholipid Syndrome/drug therapy
• Antiphospholipid Syndrome/complications
• Female
• Male
• Risk Factors
• Retrospective Studies
• Hemorrhage/chemically induced
• Hemorrhage/epidemiology
• Middle Aged
• Thrombosis/drug therapy
• Thrombosis/etiology
• Fibrinolytic Agents/adverse effects
• Fibrinolytic Agents/therapeutic use
• Fibrinolytic Agents/administration & dosage
• Anticoagulants/adverse effects
• Anticoagulants/administration & dosage
• Adult
• Warfarin/adverse effects
• Warfarin/administration & dosage
• Aged
• Thrombocytopenia/complications
• Recurrence
• Logistic Models

• anti-complement monoclonal antibodies
• atypical hemolytic uremic syndrome
• preeclampsia
• systemic lupus erythematosus
• thrombotic microangiopathy (tma)

• APS
• DVT
• anticoagulation
• antiphospholipid syndrome
• deep vein thrombosis
• thrombocytopenia

• DVT
• anticoagulation
• deep vein thrombosis
• disease
• low-molecular-weight heparin
• lower
• thrombophilia

• antiphospholipid antibodies
• antiphospholipid syndrome
• antiplatelets
• platelets
• thrombosis

• SLE
• damage
• mortality
• thrombocytopenia

• Humans
• Male
• Egypt/epidemiology
• Lupus Erythematosus, Systemic/diagnosis
• Lupus Erythematosus, Systemic/drug therapy
• Lupus Erythematosus, Systemic/epidemiology
• Antibodies, Antiphospholipid
• Risk Factors
• Thrombocytopenia/diagnosis
• Thrombocytopenia/epidemiology

• Anticoagulants
• Antiphospholipid antibodies
• Antiphospholipid syndrome
• Aspirin
• Lupus anticoagulant
• Thrombocytopenia

• Humans
• Antiphospholipid Syndrome/complications
• Anticoagulants/therapeutic use
• Antibodies, Antiphospholipid
• Thrombosis
• Thrombocytopenia/complications

• Humans
• Antiphospholipid Syndrome/complications
• Antiphospholipid Syndrome/drug therapy
• Antiphospholipid Syndrome/diagnosis
• Venous Thromboembolism/drug therapy
• Venous Thromboembolism/etiology
• Antibodies, Antiphospholipid
• Anticoagulants/adverse effects
• Lupus Coagulation Inhibitor/therapeutic use

• antibodies
• antiphospholipid
• antiphospholipid syndrome
• hydroxychloroquine

• Adult
• Humans
• Antiphospholipid Syndrome/diagnosis
• Antiphospholipid Syndrome/therapy
• Antibodies, Antiphospholipid
• Hydroxychloroquine

Pulmonary thromboembolism is a common disease frequently encountered in the emergency room and has a high mortality rate. Antiphospholipid syndrome (APS) is a high-risk factor for recurrent pulmonary embolism (PE). It is critical to effectively administer anticoagulants to avoid the recurrence of thrombotic events. This study aims to identify the clinical characteristics of APS patients with PE (APS-PE) and to develop a risk score for determining the presence of APS in PE patients in the emergency situations.

We retrospectively enrolled 76 PE patients in this study, with 46 patients in the APS-PE group and 30 patients in the non-APS-PE group. We compared differences in demographics, laboratory parameters, and early mortality risk between the two groups. Risk factors for APS-PE were screened using logistic regression analysis. We also developed an early risk score using multivariate analysis weighted points proportional to the β- regression coefficient values and calculated the sensitivity and specificity for APS in PE patients.

In the APS-PE group, we observed a higher proportion of males (43.6 vs. 20%), a higher proportion of low-risk patients (58.7 vs. 10%), lower levels of white blood cells and platelets (PLT), longer activated partial thromboplastin time (APTT), and a slight increase in D-dimer levels. Patients who were triple positive for antiphospholipid antibodies (aPLs) were younger. The APTT gradually increased as the number of positive aPLs increased. The risk factors for APS included male (OR = 5.565, 95% CI 1.176–26.341), decreased PLT (OR = 0.029, 95% CI 0.003–0.330), slightly increased D-dimer (OR = 0.089, 95% CI 0.019–0.426), and prolonged APTT (OR = 4.870, 95% CI 1.189–19.951). The risk score was named MPDA and included male, PLT, D-dimer and APTT, which can predict APS in PE patients with the AUC at 0.888 (95% CI 0.811–0.965).

The risk factors for APS in PE patients are male, low PLT, prolonged APTT and slightly increased D-dimer. The MPDA is a quantitative scoring system which is highly suggestive of APS in PE patients.

Antiphospholipid syndrome (APS) is an autoimmune response characterized clinically by arterial or venous thrombosis. One of the rare and series forms of APS is the catastrophic APS (CAPS). The incidence of CAPS has been reported in 0.8% of patients with APS. There have been very few case reports with cardiac involvement in CAPS. Common cardiac manifestations include valvular thickening and lesions, coronary artery disease, and myocardial infarction due to microvascular thrombosis. Here, we are reporting a case of CAPS associated with heart failure and a literature review of similar cases.

A 24-year-old woman with a history of APS presented with shortness of breath and right-sided pleuritic chest pain. Computed tomography pulmonary angiogram revealed new pulmonary emboli in the right lung. After 5 days, she developed high-grade fever with negative infectious workup, acute hypoxic respiratory failure with pulmonary oedema, shock, acute kidney injury, and transthoracic echocardiography showed reduced ejection fraction and global hypokinesia. The constellation of multi-organ failure, symptoms within a week, the presence of antiphospholipid antibodies, and exclusion of other causes, CAPS was diagnosed. The patient showed significant improvement with pulse steroids, IV plasmapheresis and got discharged on oral prednisone taper and anticoagulation with home health.

There are different cardiac complications associated with CAPS, including congestive heart failure, acute coronary syndrome, valvular lesions, and thrombus. Heart failure management in CAPS includes triple therapy of intravenous immune globulin, IV plasmapheresis, and corticosteroids rather than conventional treatment.

This study aims to evaluate the prevalence of thrombocytopenia in a cohort of patients with primary and secondary antiphospholipid syndrome (APS) and to examine the relation of thrombocytopenia to the clinical, laboratory findings, and damage index for antiphospholipid syndrome (DIAPS).

Between August 2018 and February 2019, a total of 168 patients (16 males, 152 females; mean age: 32.5±8.4 years; range, 18 to 59 years) who were followed in our clinic for APS were retrospectively analyzed. Medical records of the patients were screened and clinical data, laboratory investigations, and treatments applied were recorded. The DIAPS was calculated for all patients. The patients were divided into two groups according to the presence or absence of thrombocytopenia and both groups were compared regarding clinical, laboratory findings and DIAPS. Further subgroup analysis was done for patients with primary APS.

The most common clinical manifestations in our patients were obstetric manifestations (77.4% in pregnant women), musculoskeletal manifestations (69%) and peripheral vascular thrombosis (54.8%). The prevalence of thrombocytopenia in our study was 42.3%, and it was significantly associated with musculoskeletal manifestations (p=0.043), vascular thrombosis (p=0.043), neurological manifestations (p=0.030), cutaneous manifestations (p=0.006), and use of immunosuppressives (p=0.047). The DIAPS was significantly higher in the thrombocytopenia group (p=0.034). Further subgroup analysis of patients with primary APS revealed that neurological manifestations (p=0.010) were significantly higher in the thrombocytopenia group, while the DIAPS was higher in the thrombocytopenia group, but it did not reach statistical significance (p=0.082).

Thrombocytopenia may be associated with a higher incidence of vascular thrombosis, neurological manifestations, musculoskeletal manifestations, use of immunosuppressive treatment, and DIAPS. In primary APS patients, thrombocytopenia may be a risk for neurological manifestations.

• antiphospholipid syndrome
• chronic thromboembolic pulmonary hypertension
• endarterectomy
• pulmonary embolism
• pulmonary hypertension

Cohen syndrome is an autosomal recessive disorder with the primary symptoms of mental deficiency, progressive retinopathy, hypotonia, microcephaly, obesity of midchildhood onset, intermittent neutropenia, and dysmorphic facial features. The syndrome has high phenotypic heterogeneity and is caused by loss-of-function mutations in the VPS13B gene. Here, we introduce a novel homozygous nonsense mutation (c.8698G > T, p.E2900X) in the VPS13B gene in an 11-year-old Iranian boy with major symptoms of Cohen syndrome. He also had mild anemia accompanied by positive antiphospholipid antibodies, the latter has never been previously reported in Cohen syndrome.

Antiphospholipid syndrome (APS) is frequently associated with thrombocytopenia, in most cases mild and in the absence of major bleedings. In some patients with a confirmed APS diagnosis, secondary immune thrombocytopenia (ITP) may lead to severe thrombocytopenia with consequent major bleeding. At the same time, the presence of antiphospholipid antibodies (aPL) in patients with a diagnosis of primary ITP has been reported in several studies, although with some specific characteristics especially related to the variety of antigenic targets. Even though it does not enter the APS defining criteria, thrombocytopenia should be regarded as a warning sign of a “high risk” APS and thus thoroughly evaluated. The presence of aPL in patients with ITP should be assessed as well to stratify the risk of paradoxical thrombosis. In detail, besides the high hemorrhagic risk in secondary thrombocytopenia, patients with a co-diagnosis of APS or only antibodies are also at risk of arterial and venous thrombosis. In this narrative review, we discuss the correlation between APS and ITP, the mechanisms behind the above-reported entities, in order to support clinicians to define the most appropriate treatment strategy in these patients, especially when anticoagulant or antiplatelet agents may be needed.

• antiphospholipid antibodies
• antiphospholipid syndrome
• immune thrombocytopenia
• lupus anticoagulant
• thrombocytopenia

• aPL profile
• anti-platelet antibodies. systemic lupus erythematosus
• antiphospholipid syndrome
• mortality
• platelet count
• thrombocytopenia

分析伴血小板减少原发性抗磷脂综合征（PAPS）的临床特征、与血小板减少相关的危险因素以及疾病复发风险。

回顾性分析2009年至2019年间于北京协和医院住院治疗的PAPS患者，比较血小板减少（PLT<100×10⁹/L）和血小板正常患者的临床和实验室检查结果，分析血小板减少患者的临床特征和未来症状再发风险，并应用单因素和多因素Logistic回归分析筛选血小板减少的危险因素。

纳入127例PAPS患者，其中36例（28.3%）合并血小板减少，中位年龄38.0（28.5,51.5）岁，女性占63.9%（13/23），平均血小板计数为（58.9±27.0）×10⁹/L。与血小板正常组（91例）相比，血小板减少组血栓及病理性妊娠发生率差异无统计学意义（P>0.05），自身免疫性溶血性贫血（19.4%对3.3%）、网状青斑（16.7%对3.3%）、慢性肾脏病变（25%对8.8%）、抗磷脂（aPL）抗体三阳性（61.1%对37.4%）发生率均显著增加（P<0.05），补体C3、C4水平显著降低（C3 0.87 g/L对1.07 g/L，C4 0.12 g/L对0.18 g/L，P值均<0.05），中位改良APS总体评分（aGAPSS）显著升高（13分对9分，P＝0.037）。多因素Logistic回归分析显示，低补体血症（OR＝5.032，95%CI 3.118～22.095）是发生血小板减少的危险因素。

PAPS患者血小板减少多为轻中度降低。低补体血症是血小板减少的危险因素。伴血小板减少的PAPS患者再发风险较高。

• Hypocomplementemia
• Primary antiphospholipid syndrome
• Thrombocytopenia

• Antiphospholipid antibodies
• Antiphospholipid syndrome
• Systemic lupus erythematosus
• Thrombocytopenia
• Thrombosis

• Antibodies, Antiphospholipid
• Antiphospholipid Syndrome/complications
• Female
• Humans
• Lupus Erythematosus, Systemic
• Morbidity
• Pregnancy
• Thrombocytopenia
• Thrombosis

• Arterial
• Glycoprotein
• Phospholipids
• Syndrome
• Thrombosis

• Catastrophic antiphospholipid syndrome
• high-dose intravenous immunoglobulin
• microvascular
• therapeutic plasma exchange
• thrombocytopenia
• thrombosis

• Livedo reticularis
• Pregnancy morbidity
• Risk factor
• Thrombocytopenia
• Thrombosis

Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding – and persistent knowledge gaps – through clinically oriented questions and answers.

A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Although the pathophysiology underlying thrombocytopenia in APS has yet to be definitively revealed, mechanisms that play a role (at least in subsets of patients) include: immune thrombocytopenic purpura/ITP-like autoantibodies against platelet glycoproteins; antiphospholipid antibody (aPL)-mediated platelet activation and consumption; and potentially life threatening thrombotic microangiopathy. Although thrombocytopenia is often ‘mild’ in APS (and therefore, may not require specific therapy), there are causes of acute-onset thrombocytopenia that mandate emergent work-up and treatment. When APS-related thrombocytopenia does require therapy, the approach must be individualized (requiring an understanding of pathophysiology in the particular APS patient). For patients with ITP-like disease, rituximab is emerging as a popular approach to treatment; in contrast, there are hints that thrombopoietin mimetics may be associated with elevated thrombotic risk.

Thrombocytopenia is common in APS, and is likely associated with more severe disease. Improved understanding of thrombocytopenia in APS has the potential to improve risk stratification, reveal novel aspects of APS pathophysiology, and lead to treatments that are more individualized and holistic.

• Antibodies, Antiphospholipid
• Antiphospholipid Syndrome
• Humans
• Purpura, Thrombocytopenic, Idiopathic/drug therapy

• Antiphospholipid syndrome
• Arterial thrombosis
• Biologic therapy
• Catastrophic antiphospholipid syndrome
• Immunosuppressive therapy

• Beta2-glycoprotein I
• antiphospholipid syndrome
• catastrophic illness
• microcirculation
• platelet function tests

• Antiphospholipid syndrome
• anti-β2GPI antibodies
• complement
• inflammation
• microangiopathy

• Antiphospholipid syndrome
• antiphospholipid antibodies
• pregnancy morbidity
• risk assessment
• vascular thrombosis

• Antiphospholipid antibody syndrome
• Cardiolipin
• Lupus anticoagulant
• Phospholipids
• β2-Glycoprotein I