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Catastrophic antiphospholipid syndrome

Jenny Z Wang, Kourosh Beroukhim, Yi-Chun Chen, Danielle Tartar, Maxwell Fung, Alain Brassard
No abstract text is available yet for this article.
February 2019: JAAD Case Reports
Saravanan Margabandhu, Suchitra Ranjit, Indira Jayakumar, Chitra Sundaramoorthy, Mahesh Janarthanan, Jayakumar Reddy, Meena Thiagarajan, Shyamala Jayamoorthy, Latha Vishwanathan
INTRODUCTION: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients. SUBJECTS AND METHODS: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken. Plasma exchange was given as adjunctive therapy along with primary treatment for the disease concerned. Demographic and clinical data were studied, and descriptive statistical analysis was carried out...
July 2018: Asian Journal of Transfusion Science
Michelle Sholzberg
When faced with a life-threatening non-cancerous blood disorder, the term "benign" is a misnomer. Devastating diseases like catastrophic antiphospholipid antibody syndrome, acquired hemophilia, and severe immune thrombocytopenia present a challenge to the hematologist. They are often difficult to treat and lack appropriately powered, unbiased evidence to support management. Moreover, the label "benign" does a disservice as it subconsciously triggers discrepancies in prioritization for the care provider, the system, the patient and his/her family...
January 2019: Research and Practice in Thrombosis and Haemostasis
James Cranley, Unni Krishnan, Katharine Tweed, Rudolf Martin Duehmke
A 51-year-old woman with known primary antiphospholipid syndrome presented with a 4-day history of chest and abdominal pain, inferior ST-segment elevation on a 12-lead ECG and a subtherapeutic international normalised ratio. In view of a significantly raised high-sensitivity troponin I assay, inferior wall hypokinesis on transthoracic echocardiography and despite unobstructed epicardial vessels on emergency coronary angiography, a diagnosis of myocardial infarction was made. Furthermore, the patient also developed both bilateral adrenal haemorrhages leading to acute adrenal insufficiency and microvascular thrombotic renal disease concurrently...
January 10, 2019: BMJ Case Reports
Mehak Singh, Manoj Pawar
No abstract text is available yet for this article.
December 20, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
J Y Gu, C Lu, H Shi, C D Yang
OBJECTIVE: Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a special subtype of antiphospholipid syndrome (APS) characterized by multiple intravascular thrombosis involving multiple organs systems or tissues simultaneously or continuously, high titer antiphospholipid antibodies and high mortality rate. This article's aims was to analyze the clinical manifestation, laboratory examination and treatment therapy of CAPS for the purpose of improving the understanding, diagnosis and treatment of the disease in clinical practice...
December 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Huma Zahid, Saira Hassan, Salma Gul, Komal Rizwan, Saif Ullah Khan, Maaz A Maghazi
Young patients presenting with thrombotic events like pulmonary embolism and cardiological phenomenon such as presence of an intracardiac mass, without any underlying risk factors, should be promptly investigated for thrombophilias including antiphospholipid syndrome (APLS). This case is reported to highlight rare occurrence of co-existing bilateral extensive pulmonary embolism and an intra-cardiac mass at presentation of antiphospholipid syndrome as well as progression to near catastrophic APLS.
October 10, 2018: Curēus
Victoria Sadick, Stuart Lane, Eddy Fischer, Ian Seppelt, Anita Shetty, Anthony McLean
Catastrophic antiphospholipid syndrome is a rare multisystem autoimmune condition characterised by rapid development of widespread thrombotic disease and subsequent multi-organ failure. It is the most severe complication of antiphospholipid syndrome, carrying significant morbidity and mortality. We report a patient with post-partum catastrophic antiphospholipid syndrome with cardiac, hepatic, renal and cutaneous manifestations. The diagnostic challenges in establishing a definitive diagnosis in catastrophic antiphospholipid syndrome is discussed, along with the difficulties in managing these patients in the intensive care unit...
November 2018: Journal of the Intensive Care Society
John M Gansner, Nancy Berliner
Catastrophic antiphospholipid antibody syndrome (CAPS) and macrophage activation syndrome (MAS) are both life-threatening hematologic disorders that infrequently afflict patients with rheumatologic disease. CAPS is characterized by fulminant multiorgan damage related to small vessel thrombosis in the setting of persistent antiphospholipid antibodies. It can occur in patients with rheumatologic diseases such as systemic lupus erythematosus but can also affect patients who do not have rheumatologic disease. By contrast, the term MAS is applied when patients with rheumatologic disease develop hemophagocytic lymphohistiocytosis (HLH); therefore, patients with MAS have an underlying rheumatologic disease by definition...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
R Shah, S V Brodsky, L Hebert, B H Rovin, T Nadasdy, A A Satoskar
Antiphospholipid antibody syndrome (APS) is an acquired prothrombotic autoimmune disease caused by the presence of antibodies against anionic phospholipids or plasma proteins bound to phospholipids on cell membranes. It can be a primary disease or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Laboratory testing for antiphospholipid antibodies (aPL) may be only transiently positive, so APS could be missed until a catastrophic thrombotic episode or pregnancy morbidity occurs...
December 2018: Lupus
Jose Maria Morales, Manuel Serrano, Jose Antonio Martinez-Flores, Dolores Perez, Antonio Serrano
Renal allograft thrombosis is the most frequent and devastating complication in the early postrenal transplantation period. Several risk factors to develop graft thrombosis depending on donors and recipients are well known. Antiphospholipid syndrome (APS) is well recognized as an important cause of kidney injury, with specific clinical and histological features that may lead to renal injury caused by thrombosis at any location within the renal vasculature. There are 3 forms of APS, primary (the most common form), associated to other systemic autoimmune diseases (SAD-APS) and catastrophic...
October 30, 2018: Transplantation
Rosanna Satta, Gabriele Biondi
Antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition characterized by the onset of venous and/or arterial thrombosis, often multiple, and pregnancy morbidity in a background of antiphospholipid antibodies (aPL) positivity. Some patients can be carrier of aPL with no clinical symptoms, in other cases clinical manifestation can range from the classical presentation to an acute life-threatening condition named Catastrophic APS. APS can be considered as primary or associated to other disease, however pregnancy acts as a triggering factor on a susceptible background that lead to the clinical manifestations through immunological and non-immunological mechanism...
October 29, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
M Plüß, M Zeisberg, G A Müller, R Vasko, P Korsten
We report the case of a 25-year-old female patient who presented with purpura fulminans as a manifestation of primary antiphospholipid syndrome. Purpura fulminans is considered a rare cutaneous manifestation of antiphospholipid syndrome. Most frequently, it occurs in the context of catastrophic antiphospholipid syndrome and is associated with significant morbidity and mortality, either due to loss of affected extremities or thromboembolic damage to internal organs. After insufficient efficacy of parenteral anticoagulation and oral glucocorticosteroid treatment, we escalated treatment to high-dose intravenous glucocorticosteroid and five consecutive sessions of plasma exchange with good and sustained clinical response...
November 2018: Lupus
Patrizia Rovere-Querini, Valentina Canti, Roberta Erra, Esperia Bianchi, Giorgio Slaviero, Armando D'Angelo, Susanna Rosa, Massimo Candiani, Maria Teresa Castiglioni
RATIONALE: Hypercoagulability and pregnancy morbidity are hallmarks of the antiphospholipid syndrome (APS). Catastrophic antiphospholipid syndrome (CAPS) is a potentially life-threatening omplication of APS, with widespread acute thrombotic microangiopathy (TMA) that can be precipitated by pregnancy and delivery and result in multiorgan damage. Unrestrained activation of the complement cascade is involved, favoring endothelial activation, tissue factor expression by leukocytes, and platelet aggregation...
October 2018: Medicine (Baltimore)
Amelia Ruffatti, Giustina De Silvestro, Piero Marson, Marta Tonello, Antonia Calligaro, Maria Favaro, Teresa Del Ross, Ariela Hoxha, Elena Mattia, Vittorio Pengo
The study aimed to evaluate the clinical significance of laboratory findings in patients with catastrophic antiphospholipid syndrome (CAPS) and to report the effects of a well-defined treatment protocol in 14 consecutive cases. Thirteen patients (12 presenting one and one presenting two episodes of CAPS) were consecutively treated and monitored between 1986 and 2017. Antiphospholipid antibody (aPL) characteristics of the patients were compared with those of 64 matched controls (45 antiphospholipid syndrome patients and 19 aPL carriers) who did not develop CAPS during the same mean follow-up period (12 years ± 9...
September 2018: Journal of Autoimmunity
Claudia Mendoza-Pinto, Mario García-Carrasco, Ricard Cervera
PURPOSE OF REVIEW: The antiphospholipid syndrome (APS) is characterized by the development of thrombotic events and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). An infectious etiology for this syndrome has been postulated. The present review is aimed to summarize recent evidence about the role of infections and vaccines in the pathogenesis of the APS (including its catastrophic variant). RECENT FINDINGS: There is an increased risk of developing aPL in various infections, particularly in viral infections...
August 20, 2018: Current Rheumatology Reports
Myat Han Soe, Krishna Adit Agarwal, Alueshima Akough-Weir
Catastrophic antiphospholipid syndrome (CAPS) is a rare but highly fatal clinical syndrome that occurs in up to 1% of patients with antiphospholipid syndrome (APS). The diagnosis of CAPS is often delayed because its presentation with multiple organ thromboses can be confused with other thrombotic microangiopathies and severe sepsis. We report a case of CAPS in a patient with APS and systemic lupus erythematosus (SLE) presenting with thrombotic storm precipitated by trauma, cytomegalovirus (CMV) infection, and noncompliance with anticoagulation therapy...
2018: Case Reports in Medicine
Mathilde Guillot, Cedric Rafat, David Buob, Paul Coppo, Matthieu Jamme, Eric Rondeau, Olivier Fain, Arsène Mekinian
No abstract text is available yet for this article.
November 1, 2018: Rheumatology
Zakaria Hindi, Mosa Hussein, Abdallah Gad, Abdallah A Khaled, Talal Zahoor
Catastrophic antiphospholipid syndrome is a rare disorder that remains under-recognized causing a high mortality rate even with treatment. Factors such as infections and systemic lupus erythematosus flare play as an inciting event in the thrombotic crisis which underlies catastrophic antiphospholipid syndrome. The use of plasmapheresis has improved the outcome of such cases with a reduction in mortality rate from over 50% to less than 30%, according to some studies. However, the definitive treatment of this disabling and fatal condition remains an area warranting research...
2018: SAGE Open Medical Case Reports
Mohamed Momtaz, Ahmed Fayed, Khaled Marzouk, Amr Shaker
Therapeutic plasma exchange is used in treating different immunological and non-immunological diseases. We analyzed the outcome of 308 patients treated by 1783 membrane plasma exchange sessions from January 2011 until January 2017 at Cairo University Hospital. Thrombotic microangiopathies were the commonest indication [73 (23.7%) patients] with response in 63/73 patients (86.3%), followed by systemic vasculitis with pulmonary-renal involvement [40(13%) patients] with recovery in 32/40 patients (80.0%), Guillain-Barré syndrome [39(12...
December 2018: Therapeutic Apheresis and Dialysis
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