Case Of Antiphospholipid Antibody Syndrome Research Articles

Perinatal outcomes in indian women with Antiphospholipid Antibody Syndrome (APS): Five year experience from a tertiary care centre

BackgroundAntiphospholipid Syndrome (APS) is a systemic autoimmune thrombophilic condition characterized by obstetric manifestations, including pregnancy loss, preeclampsia and fetal growth restriction. Early diagnosis and management are key to improve maternal and neonatal outcomes. ObjectiveThe aim of this study is to assess the perinatal outcomes in APS, the development of various adverse pregnancy outcomes (APO), and their association with specific antibody profiles. Material methodsThis observational study was carried out on booked cases of singleton pregnancy and diagnosed cases of primary APS in our High-Risk Pregnancy (HRP) clinic from January 2018 to December 2022 after approval from institutional ethics committee. Forty-three confirmed cases of primary APS were enrolled and started on low-dose aspirin and low-molecular-weight heparin (LMWH) as per the patient's body weight after confirmation of fetal heart activity radiologically until 36 weeks of gestation as a standard of care. ResultsForty patients (93 %) had obstetric APS, and three patients (7 %) had thrombotic APS. During the course of the current pregnancy, adverse pregnancy outcomes (APO) developed in 12 (30 %) out of 40 cases of obstetric APS and in all 3 patients with thrombotic APS. Preeclampsia was seen in 11 (25.5 %), FGR in 12 (27.9 %), and preterm birth in 7 (16.2 %) cases. Patients with an antibody profile showing the presence of Anti-β2 GP-I positivity and ACL positivity had fewer APOs (20 % and 29 %) in comparison to patients with a LA and triple positive antibody profile (55 % and 50 %). ConclusionTreatment of pregnant women with APS causes significant improvement in the live birth rate. The late pregnancy complications like preeclampsia, FGR, and premature birth, occurring despite treatment still remains a challenge and emphasizes the need for stringent antepartum surveillance and timely delivery.

Delayed-type hypersensitivity reaction to enoxaparin in a pregnant woman

A 32-year-old pregnant woman (G5P0L0A4) presented at 10+ weeks of gestation with multiple itchy plaques of 3 weeks duration. She was a known case of antiphospholipid antibody syndrome and was...

Intra-cardiac thrombus in antiphospholipid antibody syndrome: An unusual cause of fever of unknown origin with review of literature

Classically, antiphospholipid antibody syndrome (APS) presents with recurrent episodes of vascular thrombosis and abortions. For APS to present as fever of unknown origin (FUO) is a rare phenomenon. We present an interesting case of FUO who on workup was found to have primary APS with right atrial thrombus and chronic pulmonary thromboembolism (PTE). Fever resolved completely with anticoagulation therapy and surgical removal of the intra-cardiac thrombus. Although rare, APS should be considered in any case of FUO with prolonged activated partial thromboplastin time and/or thrombocytopenia. We also take this opportunity to briefly review 28 cases of APS with intra-cardiac thrombus reported to date in the medical literature.<Learning objective: Primary antiphospholipid antibody syndrome (APS) presenting as fever of unknown origin (FUO) is rare. APS should be kept in the differential diagnosis in any case of FUO with prolonged activated partial thromboplastin time or thrombocytopenia. Intra-cardiac thrombus is more frequently associated with primary APS as compared to secondary APS.>

Lupus anticoagulant testing using two parallel methods detects additional cases and predicts persistent positivity

Antiphospholipid antibody syndrome (APS) is characterized by laboratory evidence of antiphospholipid antibodies (aPL) [e.g. lupus anticoagulant (LA), anticardiolipin (ACL), and/or antiβ2-glycoprotein I (aB2GPI)] in a clinical setting of thrombosis or pregnancy morbidity. The International Society on Thrombosis and Haemostasis recommends two different testing modalities to detect LA. To evaluate these recommendations in a clinical setting, our hospital, a tertiary care center with a specialized coagulation laboratory, added the dilute Russell's viper venom time to be performed in parallel with the PTT-lupus anticoagulant to detect LA. Results of aPL testing were collected on all patients who had LA testing for one year. Chart review was performed to correlate LA results with ACL, aB2GPI, and clinical history. Patients who were initially LA positive by both PTT-lupus anticoagulant and dilute Russell's viper venom time were more likely to be persistently positive. Patients who were positive for ACL and aB2GPI were likely to be positive by both LA methodologies. No single method was absolutely sensitive, as cases of APS were detected by PTTLA only, DRVVT only, and both methods. The addition of a second testing method for LA provides additional diagnostic information and may be helpful in stratifying risk of thrombosis.

抗凝固療法中に下腿潰瘍と脳梗塞を繰り返し 抗リン脂質抗体の関与が疑われた 1 例

抗凝固療法中に下腿潰瘍と脳梗塞を繰り返し 抗リン脂質抗体の関与が疑われた 1 例

Investigation of Transverse Myelitis in Systemic Lupus Erythematosus Patients, a Taiwanese Experience

Objective: Transverse myelitis (TM) is one of the neurological manifestations of systemic lupus erythematosus (SLE). It can bring about long-term neurological sequelae and even mortalities. However, only a few studies have described its characteristics. This study provided data in Taiwanese Han SLE patients with TM.Methods: We recruited 12 SLE patients with 17 episodes of transverse myelitis from July 2002 to June 2012 and reviewed their medical records. Demographic data, clinical characteristics, laboratory findings and radiographic features were obtained. Severity of TM was expressed in Expanded Disability Status Scale (EDSS) scoring.Results: The median age of the 12 patients (two males and ten females) was 34 years. Among the 17 TM episodes, there was a greater than 3-point change in SLEDAI score in 64.7%, indicating a lupus flare. Most of patients had either elevated cerebrospinal fluid IgG index or protein. Involvement of more than 4 segments in spinal MRI was observed in most episodes (83.3%) of TM. Antiphospholipid antibodies were detected in only 8.3% of our patients and no case of antiphospholipid antibody syndrome was documented. More severe disease was associated with increased white blood cell count in cerebrospinal fluid and thrombocytopenia, manifested concomitant with or before the episode of TM. There was only modest improvement after treatment 6 months later in terms of EDSS scores.Conclusion: Among the Taiwanese Han population, TM is similar to that in Caucasians in many aspects. Generalized lupus flare is often present. Antiphospholipid antibodies may play a minor role in TM pathogenesis in Taiwanese SLE patients, in contrast to Caucasians with SLE-related TM.

A young case of antiphospholipid antibody syndrome, with echogenic plaque observed by intravascular ultrasound in acute myocardial infarction

A young case of antiphospholipid antibody syndrome, with echogenic plaque observed by intravascular ultrasound in acute myocardial infarction

Antiphospholipid antibody syndrome and pregnancy

Recurrent foetal loss in early and late stages of pregnancy is a common problem of women affected by anti-phospholipid antibody syndrome. The therapeutic scheme consisting of associating aspirin and heparin has shown an improvement of the prognosis for the next pregnancies. We report 3 cases of anti-phospholipid antibody syndrome and pregnancy, and we underline the clinical, therapeutic and prognosis features of this syndrome.

Progression of antiphospholipid antibody syndrome to catastrophic antiphospholipid antibody syndrome acutely with cessation of antithrombotic therapy

Catastrophic antiphospholipid antibody syndrome (CAPS) is a serious condition that is often unrecognised with a high mortality. Cessation of anticoagulation in antiphospholipid antibody syndrome (APS) can have devastating consequences with progression to CAPS. Making a diagnosis of APS can however be challenging because of the evolving diagnostic criteria and difficulty in confirming thromboses. Management of these patients can also be complex, especially in those with coexistent thrombocytopenia. New potential treatments are emerging targeted on the immunomodulation of APS rather than just prevention of thrombosis. This article aims to highlight these diagnostic and management difficulties by reporting and discussing three cases of APS with progression to CAPS following cessation of anticoagulation, one with fatal consequences, with confirmation of CAPS on autopsy, and two with successful treatment and outcomes.

A Case of Secondary Antiphospholipid Antibody Syndrome with Thyroid Cancer

Antiphospholipid antibody syndrome (APS) is defined as the presence of lupus anticoagulant antibody or anticardiolipin antibody with vascular thrombosis or pregnancy complications. APS can be associated with autoimmune disease or infectious disease. APS has also been reported in conjunction with variety of solid and hematologic malignancies. There were some reports on APS which were accompanied by hematologic malignancy, but there was no report with solid malignancy in Korea. We experienced one case of secondary APS, which was diagnosed during pre-operative evaluation of thyroi d cancer. This patient had prolonged aPTT (activate partial thromboplastin time) and decreased coagulation factors which were regarded as hemophilia at first. Although the precise mechanism of the relationship between APS and cancer has not been proven thoroughly, APS can be accompanied by various malignancies. So proper screening and early detection of malignancies in APS patients are recommended. Key Word. Antiphospholipid antibody syndrome, Malignancy, Thyroid cancer

The obstetric outcome following treatment in a cohort of patients with antiphospholipid antibody syndrome in a tertiary care center

Antiphospholipid antibody syndrome (APAS) is regarded as the most frequently acquired risk factor for thrombophilia. The obstetric manifestations of APAS include early or late pregnancy losses and complications like preeclampsia and fetal growth restriction. Its timely diagnosis and treatment can improve maternal and neonatal outcome. To study the pregnancy outcome of patients with APAS treated with heparin and aspirin. This was a retrospective study of pregnancy outcome in 42 consecutive women with APAS, treated with heparin and aspirin. The case records of 42 diagnosed cases of APAS with pregnancy, over a 3-year period, were studied. The pregnancy outcome in this group was compared before and after treatment with heparin and low-dose aspirin in terms of abortions, intrauterine deaths and live birth rate. The outcome of the present pregnancy in terms of fetal and maternal complications was analyzed. The mean age and average parity of women with APAS were 30.1±4.1 years and 3.2±1.2, respectively. Among the treated patients of APAS, 13 (30.9%) had preeclampsia and 9 (21.4%) had intrauterine growth restriction (IUGR). There were 2 (4.7%) intrauterine deaths, 4 (9.5%) missed abortions and 3 (7.1%) abruptio placentae. Women with APAS had a live birth rate of 4.6% before treatment and 85.7% in the index pregnancy after treatment. Treatment of pregnant women with APAS results in marked improvement in the live birth rate (4.6-85.7%). However, complications like preeclampsia and IUGR occur even after treatment, requiring strict monitoring and timely delivery.

A Case of Anti-phospholipid Antibody Syndrome

A Case of Anti-phospholipid Antibody Syndrome

Successful plasma exchange combined with rituximab therapy in aggressive APS-related cutaneous necrosis

Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by venous and/or arterial thrombosis or recurrent fetal loss associated with the presence of antiphospholipid antibodies and/or a lupus anticoagulant. The skin appears to be an important target organ and many cases of APS may present with skin manifestations. These lesions may be manifold and may take the form of livedo reticularis, livedo racemosa, ulcerations, digital gangrene, subungeal splinter hemorrhages, superficial venous thrombosis, thrombocytopenic purpura, pseudovasculitic manifestations, extensive cutaneous necrosis, or primary anetoderma. We report a case of fulminant APS-related cutaneous necrosis. A 38-year-old Caucasian male with a past history of APS, multiple deep vein thrombi/pulmonary emboli, presented with necrotic lesions on his right upper and right lower extremities. Initially, baseline anticoagulation was increased without improvement. Subsequently, plasma exchange was initiated on a daily schedule. Furthermore, rituximab 1,000 mg IV was administered on days 1 and 15. After six consecutive daily sessions of plasma exchange, there was significant regression of the necrotic lesions. After a 22-day hospital stay, the patient was discharged to home on fondaparinux. The patient presented approximately 2 months later after missing follow-up appointments. At the time, his initial lesions looked remarkably improved.

A case of antiphospholipid antibody syndrome that manifested in the course of basal cell carcinoma

A case of antiphospholipid antibody syndrome (APS) is presented, which manifested 5 years after onset of basal cell carcinoma (BCC). There were multiple collateral veins due to portal vein thrombosis. Because immunological abnormalities including anti-cardiolipin beta(2) glycoprotein-I antibody improved after surgical resection of BCC, it is likely that APS had occurred as a paraneoplastic syndrome with BCC. This case suggests that it is necessary to investigate the presence of APS when BCC is complicated by some coagulopathies.

Antiphospholipid antibody syndrome presenting with disseminated bleeding and spinal subdural hemorrhage

Case report. To report a rare cause of spinal subdural hematoma. A tertiary care university hospital in Andhra Pradesh, India. Detailed evaluation and reporting of a case admitted in the hospital. A case of antiphospholipid antibody syndrome (APS) was found to present with spontaneous spinal subdural hematoma and paraplegia. This is a very rare presentation of APS. This article describes a rare case of APS presenting with disseminated bleeding and spontaneous spinal subdural hematoma, resulting in paraplegia.

Cutaneous manifestations associated with antiphospholipid antibodies.

Primary Antiphospholipid Antibody Syndrome (PAAS) is characterized by detection of antiphospholipid antibodies associated with venous or arterial thrombosis and/or miscarriages by patients with no other associated disease such as systemic lupus erythematosus (SLE). Primary Antiphospholipid Antibody Syndrome has many clinical manifestations of which dermatological ones are probably the most common. The purpose of this study was to determine the frequency of each cutaneous lesion, describing clinical features essential for diagnosis, in patients with Antiphospholipid Antibody Syndrome (AAS) attending Walter Cantídio University Hospital. Sixty patients with clinical findings suggestive of AAS were screened, and submitted for clinical and laboratory evaluations including lupus anticoagulant (KCT), anticardiolipin antibodies (IgG and IgM: ELISA), routine laboratory tests and screening tests for possible associated conditions. Twenty-five cases of primary and 14 cases of secondary AAS were diagnosed by clinical and laboratory evidences. Persistent elevated antiphospholipid antibodies without history of thromboembolic events or miscarriages were demonstrated in 21 patients. Forty percent of the patients with AAS had a cutaneous feature as the major complaint. These were dermographism (15), acrocyanosis (13), urticaria (9), diffuse alopecia (9), livedo reticularis (seven), Raynaud's phenomenon (three), purpura (two), ulcers and necrosis (four), nodules (four), pterygium ungueum (one) and subungual hemorrhage (one). Dermatological complaints are very frequent in patients with AAS and may be the first clue to the syndrome. Therefore a careful history and detailed physical examination are essential to diagnose AAS. All dermatologists should investigate the possibility of AAS when facing cutaneous findings related to venous or arterial thrombosis or microthrombosis.

A Case of Antiphospholipid Antibody Syndrome Accompanied by Valvular Heart Disease and Moya Moya Syndrome

A case of antiphospholipid antibody syndrome, accompanied by valvular heart disease and Moya moya syndrome, has never been reported. Here, we report on a case that had mitral regurgitation and Moya moya syndrome, associated with antiphospholipid antibody syndrome secondary to systemic lupus erythematosus. This patient underwent a mitral valve replacement for mitral valve regurgitation. The postoperative course was uneventful, and the pathological findings of the mitral valve showed a degenerative change, due to chronic inflammation, a proliferative fibrous change and calcification, but without thrombus formation. However, the patient returned to the hospital with a cerebral hemorrhage, which was caused by Moya moya syndrome. Surgical drainage was performed, and the patient was discharged without any complications. The patient is on anticoagulation and immunosuppression drugs, with no problems to date. (Korean Circulation J 2003;33 (7):620-624)

A case of antiphospholipid antibody syndrome with arterial embolic episodes

A case of antiphospholipid antibody syndrome with arterial embolic episodes

A Case of Antiphospholipid Antibody Syndrome Diagnosed after Thrombosis of an Arteriovenous Shunt

A 32-year-old male dialysis patient with lupus nephritis was admitted because of shunt obstruction. The arteriovenous fistula was reconstructed, but obstruction recurred twice within several hours after surgery. A high blood level of anticardiolipin beta2-glycoprotein I antibody suggested that shunt obstruction was caused by a thrombotic tendency related to the antiphospholipid antibody syndrome. Accordingly, for the third shunt procedure, antiplatelet therapy (which had been commenced for systemic lupus erythematosus) was combined with dalteparin sodium from before surgery and warfarin was added postoperatively. This regimen prevented shunt obstruction. In conclusion, hemodialysis patients who suffer repeated shunt obstruction should be examined for antiphospholipid antibody syndrome.

A case of primary antiphospholipid antibody syndrome showing vegetation on the mitral valve through echocardiography

Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLB) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.