Probable Catastrophic Antiphospholipid Syndrome Research Articles

Suspected COVID-19 Immunization-Induced Probable Catastrophic Antiphospholipid Syndrome

In this report, we describe the case of a woman with suspected COVID-19 immunization-induced probable catastrophic antiphospholipid syndrome. The patient is a 35-year-old female with a past medical history significant for antiphospholipid syndrome, not on anticoagulation, who presented with a 5-day history of abdominal pain and distention, nausea, vomiting, and shortness of breath. She had received her first dose of the Pfizer COVID-19 vaccine one day prior to the onset of symptoms. After extensive workup at an outside hospital, she was found to be in acute heart failure exacerbated by severe mitral and tricuspid regurgitation. She was transferred to our hospital for escalation of care. EKG showed evidence of prior inferior and septal myocardial infarction. Transesophageal echocardiogram (TEE) showed reduced ejection fraction, severe mitral and tricuspid regurgitation, and a left ventricular thrombus. Cardiac MRI showed subendocardial late gadolinium enhancement indicative of ischemia. However, CTA of the coronary vessels showed no signs of obstruction. Therefore, her acute heart failure was thought to be due to small vessel thrombosis secondary to antiphospholipid syndrome. During admission, she had several absence seizure-like episodes. CT head showed several hypodensities of the deep white matter and brain MRI demonstrated multiple hyperintense T2 FLAIR signal foci with restriction diffusion and enhancement involving the cerebral hemisphere, consistent with subacute strokes attributed to being secondary to antiphospholipid syndrome or embolic from the left ventricular thrombus. She was treated with heparin for suspected catastrophic antiphospholipid syndrome and high-dose corticosteroid therapy for concomitant Systemic Lupus Erythematosus (SLE). She was discharged in a stable condition.

The challenges in diagnosing pediatric primary antiphospholipid syndrome

Pediatric primary antiphospholipid syndrome (APS) is a very rare disease with significant distinctions from the APS in adults. Herein, we present our experience in the diagnosis and treatment of six pediatric primary APS patients, who met the updated Sapporo criteria for the APS diagnosis. One of them was also diagnosed as having probable catastrophic APS (CAPS) due to the involvement of three different organ systems simultaneously. Besides vascular involvement, four patients had thrombocytopenia, one had psychiatric disorder, and one had chorea and valvular heart disease. All patients received immunosuppressive treatment along with long-term anticoagulation therapy. Specific neurologic and hematologic manifestations that are not part of the classification criteria can be seen in children with primary APS. Therefore, using the adult criteria for diagnosing pediatric APS may result in missed or delayed diagnoses in children.

A retrospective review of antiphospholipid syndrome from a South Asian country.

Objectives This study aims to investigate clinical presentations, antiphospholipid antibody patterns and their levels, therapeutic regimens, and outcomes in patients with antiphospholipid syndrome (APS) admitted to a tertiary care hospital of a South Asian country.Patients and methods Between January 2009 and December 2019, a total of 216 patients with APS (8 males, 208 females; median age: 35.7±6.9 years; range, 20 to 76 years) who either fulfilled the modified Sydney criteria or those who satisfied only clinical criteria along with positive antiphospholipid antibody on at least one occasion (probable APS) were retrospectively analyzed.Results The majority of the patients (n=183, 84.7%) had obstetric complications, followed by venous thrombosis in 23 (10.8%) patients. Recurrent early abortions in 126 (58.6%) and deep venous thrombosis in 16 (7.4%) patients were the most prevalent obstetrical and venous events, respectively, whereas limb gangrene in seven (3.3%) and ischemic stroke in seven (3.3%) were the most common arterial events. A total of 190 (88%) patients had primary APS, while 26 (12%) had secondary APS. Systemic lupus erythematosus was the frequent association with secondary APS found in 19 (73%) patients. Immunoglobulin M (IgM) anticardiolipin antibody was present in 173 (65.0%) patients, being the most commonly reported antibody. Probable catastrophic APS was found in four (1.9%) patients. Majority of the patients (n=190, 87.9%) were treated with a combination of acetylsalicylic acid and low-molecular-weight heparin. Single mortality was observed in our study population due to complications related to catastrophic APS.Conclusion Antiphospholipid syndrome has a wide range of thrombotic and obstetrical manifestations with important variations in different regions of the world. There is a significant morbidity and mortality related to APS, despite treatment with anticoagulation and; therefore, describing prognostic markers and optimal therapeutic interventions is pivotal to prevent complications.

Probable Catastrophic Antiphospholipid Syndrome Mimicking Severe Sepsis after Digital Amputation

Antiphospholipid syndrome (APLS) is an autoimmune disorder characterised by veno-arterial thrombosis, adverse outcomes in pregnancy and raised titres of antiphospholipid antibodies. The catastrophic variant is rare and makes up less than 1% of cases but carries a high mortality. We present a case of probable catastrophic antiphospholipid syndrome (CAPS) presenting shortly after toe amputation under general anaesthesia.

Probable catastrophic antiphospholipid syndrome complicated with primary sclerosing cholangitis

Probable catastrophic antiphospholipid syndrome complicated with primary sclerosing cholangitis

Recurrence of Cutaneous Necrosis in an Infant with Probable Catastrophic Antiphospholipid Syndrome

We present the case of a 3-month-old child with probable catastrophic antiphospholipid syndrome who, after initial successful management with immunomodulary therapies including rituximab, experienced a cutaneous relapse. This rare event was successfully re-treated with repeated administration of rituximab, supporting its role in the control of this disorder. Dermatologic manifestations may be the main clinical presentation of antiphospholipid syndrome, a possible underdiagnosed but potentially fatal pathology.

Severe Abdominal Pain as a Presenting Symptom of Probable Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (APS) in pediatric medicine is rare. We report 3 adolescents who presented with acute onset of severe abdominal pain as the first manifestation of probable catastrophic APS. The 3 patients, 2 male patients and 1 female patient were 14 to 18 years old. One had been diagnosed with systemic lupus erythematosus in the past, but the other 2 had no previous relevant medical history. All presented with excruciating abdominal pain without additional symptoms. Physical examination was noncontributory. Laboratory results were remarkable for high inflammatory markers. Abdominal ultrasonography was normal, and abdominal computed tomography scan showed nonspecific findings of liver infiltration. Only computed tomography angiography revealed evidence of extensive multiorgan thrombosis. All patients had elevated titers of antiphospholipid antibodies. The patients were treated with full heparinization, high-dose steroids, and intravenous immunoglobulin with a resolution of symptoms. One patient was resistant to the treatment and was treated with rituximab. In conclusion, severe acute abdominal pain can be the first manifestation of a thromboembolic event owing to catastrophic APS even in previously healthy adolescents. Diagnosis requires a high index of suspicion with prompt evaluation and treatment to prevent severe morbidity and mortality.

Acute Kidney Injury Associated with Disseminated Intravascular Coagulation that Developed after a Hysterectomy in a Patient with Probable Catastrophic Antiphospholipid Syndrome

Acute kidney injury associated with disseminated intravascular coagulation (DIC) and a coagulation factor defect may develop with infection, malignancy, severe trauma, or obstetric complications. However, a gynecological etiology, such as hysterectomy, in a patient with adenomyosis has rarely been reported. We describe a case of a 42-year-old women who presented with DIC and acute kidney injury after a total hysterectomy. We thought that the cause for the acute kidney injury might be small vascular thrombosis and acute tubulointerstital nephritis due to probable catastrophic antiphospholipid syndrome with DIC. The patient was successfully treated with steroid therapy. (Korean J Med 2012;82:739-743)

Infant With Probable Catastrophic Antiphospholipid Syndrome Successfully Managed With Rituximab

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by the presence of autoantibodies to a variety of phospholipids and phospholipid-binding proteins. Clinical manifestations range from being asymptomatic to having imminently life-threatening events. Catastrophic antiphospholipid syndrome (CAPS) occurs in <1% of patients with APS and is defined by multiple small-vessel occlusions that lead to multiple-organ failure and is associated with high morbidity and mortality rates. Here we report the case of a 3-month-old boy with probable CAPS who presented to us with digital necrosis and pulmonary hemorrhage. In addition, a skin biopsy demonstrated multiple small-vessel thromboses without signs of vasculitis. Results of testing for autoantibodies were positive for anti-beta(2) glycoprotein I (anti-beta(2)-GPI) only. His treatment consisted of high-dose steroids, immunoglobulin therapy, exchange transfusion, cyclophosphamide, and rituximab as well as iloprost and bosentan as vasodilators for his ischemia; he showed an excellent clinical response. To the best of our knowledge, this is the youngest patient with probable CAPS, the first reported patient to test positive for anti-beta(2)-GPI antibodies and negative for anticardiolipin antibodies and lupus anticoagulant, and the second patient reported to be successfully treated with an immunomodulatory regimen including rituximab.

Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature)

We describe a 58-year old female patient with rapid development of arterial and venous thromboembolisms, including deep vein thrombosis (DVT) in the lower limbs, recurrent cerebral infarctions and bilateral pulmonary emboli. Her laboratory data on admission showed positive anticardiolipin antibody of IgG isotype (IgG aCL) and positive anti-beta2 glycoprotein-I antibody of IgG isotype (IgG abeta2-GPI), and decreased protein C activity and protein S antigen. Systemic examinations revealed the presence of an ovarian cancer. Surgical resection was attempted, but her cancer infiltrated the pelvic wall and could not be resected. Despite treatment with unfractionated heparin followed by warfarin, she died due to recurrent episodes of cerebral infarction. This case was considered as probable catastrophic antiphospholipid syndrome (CAPS), which might be associated with ovarian cancer. Known as Trousseau's syndrome, arterial and, more commonly, venous thrombosis is a frequent complication of cancer and sometimes a harbinger of occult cancer. Our case indicates that there is an overlap between antiphospholipid syndrome (APS) and Trousseau's syndrome. It is important to bear in mind that a thrombotic event associated with cancer can be the first manifestation of CAPS.