Catastrophic antiphospholipid syndrome

Catastrophic APS is a potentially life-threatening condition with a high mortality, which requires high clinical awareness. New mechanisms for its production can only be explored if samples are obtained, stored, and dis-patched to investigation sites in Europe (Barcelona), the United States (Atlanta), and Japan (Sapporo). Details will be posted and made available on the International Registry Web Site in 2006. It is essential that the condition be diagnosed early and treated aggressively. The combination of high doses of iv heparin, iv steroids plus repeated doses of iv gammaglobulins or plasma exchange is the treatment of choice in patients with this severe condition. Additionally, preventive measures in patients with APS may be effective if the development of the catastrophic APS is to be avoided.     

Catastrophic antiphospholipid syndrome

Antiphospholipid syndrome (APS) is a thrombotic disorder associated with autoantibodies that target membrane phospholipids and phospholipid-binding proteins, which regulate coagulation. APS is usually characterized by major arterial or venous occlusions, pregnancy complications, or both. In 1992, Asherson described an unusual variant of APS termed the catastrophic antiphospholipid syndrome (also known as Asherson's syndrome), the hallmark of which is rapid multiorgan failure caused by widespread small-vessel thrombi. Empiric treatments have improved the prognosis of patients, but half still die from thrombotic diathesis, even though those who survive the acute stages frequently remain well. Given the persistently high mortality rate, efforts have been underway to facilitate early diagnosis, institute effective treatments in a timely manner and to better understand the cause (or causes) of this extreme condition in order to improve outcomes.     

Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome is a life-threatening subset of antiphospholipid syndrome. It is defined by the involvement of at least three different organ systems over a period of days or weeks. This review focuses on the basic pathobiology and current concepts in the management of this rare but important disorder.     

Catastrophic antiphospholipid syndrome

In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs. The thrombocytopenia is usually marked, and a Coombs positive microangiopathic-type anemia may accompany the condition. Features of disseminated intravascular coagulation may be evident in some patients. It is fatal in approximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenous corticosteroids, to offset the systemic inflammatory response syndrome that occurs as a result of the extensive tissue damage, and plasmapheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected.     

Catastrophic antiphospholipid syndrome after ovulation induction

INTRODUCTION: The catastrophic antiphospholipid syndrome (CAPS) is a rare expression of the antiphospholipid syndrome (APLS). The all predicting factors of happening of CAPS are not yet discovered. Some women presenting an APLS with infertility have recourse to ovulation induction (OI). EXEGESIS: After an OI, a woman is admitted for acute renal failure, hypertension, seizures, respiratory failure. History and immunologic tests are in favour of APLS. Renal biopsy highlights a thrombotic microangiopathy. Diagnosis of CAPS is made. Patient improves after respiratory assistance, parenteral treatment for hypertension and anticoagulant therapy. CONCLUSION: IO seems to be on of the factors which leads to CAPS. To avoid such consequences, it's essential to know the history and the immunological status of the patients having recourse to this treatment to take care of these risked pregnancies.     

Catastrophic antiphospholipid syndrome triggered by trauma.

We describe the first case of catastrophic antiphospholipid syndrome triggered by trauma. In contrast to reports that emphasize the devastating nature of the syndrome, our patient's course is less dramatic and more elusive. A possible pathophysiological explanation to the association of antiphospholipid syndrome and trauma is discussed.     

Catastrophic antiphospholipid syndrome complicating orthotopic liver transplantation

Catastrophic antiphospholipid syndrome (CAPS) is an acutely devastating situation characterized by widespread thrombotic microangiopathy in the presence of elevated titers of antiphospholipid antibodies. We describe a 57-year old woman who underwent liver transplantation for primary sclerosing cholangitis and developed this malignant variant of the antiphospholipid syndrome.     

Catastrophic antiphospholipid syndrome masquerading as ischaemic colitis

We describe a young woman whose initial presentation was dominated by acute diarrhoea. Life-threatening multiorgan failure rapidly ensued and necessitated mechanical ventilation and dialysis treatment. An initially elongated activated partial thromboplastin time prompted further coagulation tests that led to the detection of positive lupus anticoagulant, a highly elevated IgG-anticardiolipin (aCL) antibody titre, and prolonged dilute Russell's viper venom time. Histological examination of samples obtained during endoscopy revealed widespread intestinal thrombotic microangiopathy. In view of these serologic and histologic features, a diagnosis of the malignant variant of the antiphospholipid syndrome (APS), also termed 'catastrophic APS', was established. In spite of this syndrome's grim prognosis, the patient recovered following intensive anticoagulation and adjunct treatment with steroids and immunoglobulins.     

Catastrophic manifestation of the antiphospholipid syndrome.

We describe a young woman who displayed the "malignant" variant of the antiphospholipid syndrome (APS), also known as the "catastrophic APS." Renal insufficiency, retinopathy, cerebral infarcts, bone marrow necrosis, skin ulcers, and nasal septum perforation were the result of widespread thrombotic microangiopathy. She recovered during high intensity anticoagulation.     

Catastrophic antiphospholipid syndrome in the immediate puerperium

We describe a female previously diagnosed of primary antiphospholipid antibody syndrome who presented a preclampsia in the second pregnancy. An urgent caesaria was made because of a worsening high blood pressure and oliguria. In the immediated puerperium she showed low platelets and persistent high blood pressure. Afterwards acute renal failure and neurological signs with a severe aortic valvulopathy were diagnosed. An haemolytic anemia was also detected. Definitive diagnosis was made by kidney biopsy with the result of a thrombotic microangiopathy. Treatment with low weight heparin and aspirin and systemic corticosteroids was started in the immediate puerperium and fresh frozen plasma was then added with a good response to treatment. Actually she is still with high blood pressure, aortic valvulopathy. Renal function is normal one year later.     

Catastrophic antiphospholipid syndrome in a 7-year-old girl

Antiphospholipid syndrome (APS) has been recognized as the leading cause of vascular thrombosis in children. The syndrome may occur in isolation or in association with an underlying systemic disease, particularly systemic lupus erythematosus. Less than 1% of patients with APS present with a life-threatening condition resulting from thrombosis in multiple organs and subsequent multiorgan failure, which is defined as catastrophic APS. Early recognition of APS is essential because prompt and appropriate management can result in favorable outcome. We present the case of a 7-year-old girl with APS who presented with cerebral, femoral, and renal involvement in the second week of the disease progress. The patient presented with multiple thrombotic episodes and rapidly progressive renal failure. Renal cortical infarction was diagnosed by magnetic resonance imaging.     

Catastrophic antiphospholipid syndrome related to severe ovarian hyperstimulation

Antiphospholipid syndrome (APS) is a cause of infertility and fetal loss. Ovarian stimulation can induce previously unknown APS. Ovarian hyperstimulation syndrome (OHS) is uncommon but potentially life-threatening, as well as catastrophic APS. A woman that simultaneously developed a severe OHS and a catastrophic APS is described in this paper. Both entities produced thrombotic cardiac and brain thrombosis. A peculiar mechanism of cardiac ischemia is also described. In spite of the life-threatening risk of this situation, the indication for preventive anti-aggregation and/or anticoagulation is not clear.     

Catastrophic antiphospholipid syndrome: The current management approach

The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab. The first blocks CD20, a surface protein expressed on the cytoplasmic membrane of B cells, and decreases the generation of pathogenic autoantibodies such as antiphospholipid (aPL) antibodies. The second binds with high affinity to C5 complement protein, inhibiting its cleavage and thus preventing the generation of C5b–C9 complex.     

Catastrophic exacerbation of antiphospholipid syndrome after lung adenocarcinoma biopsy

We describe a 60-year-old man with nephrotic syndrome due to a glomerular thrombotic microangiopathy caused by the antiphospholipid syndrome (APS) associated with a lung adenocarcinoma. Although no significant aggravation of APS was noted following renal biopsy, catastrophic exacerbation of APS occurred 3 days after a lung adenocarcinoma biopsy while warfarin and prednisolone were being administered. The patient died of multiple organ failure 37 days after the lung adenocarcinoma biopsy. This case emphasizes the need for great caution for catastrophic exacerbation of malignancy associated APS following biopsy of the underlying malignancy.