Successful outcome with eculizumab treatment in a patient with antiphospholipid syndrome presenting with an unusual thrombotic storm.

Catastrophic Antiphospholipid Syndrome (CAPS) is a life-threatening complication of APS requiring complex management to optimize patient outcome. We describe a 54-year-old man with APS with history of splanchnic vein thrombosis, a Factor II G20210A heterozygote, autoimmune hemolytic anemia and thrombocytopenia. He developed sudden onset of severe flank pain due to spontaneous bilateral adrenal hemorrhage while on warfarin with a therapeutic INR. Despite unfractionated heparin and initial clinical improvement, severe thrombocytopenia developed requiring dexamethasone, rituximab, and romiplostim. Hospitalization was complicated further by thrombosis of the inferior vena cava, pulmonary embolism, and painful violaceous patches on his neck and ear cartilages. Punch biopsy of lesions revealed C5b-C9 deposition of small vessel thromboses. Although the inciting event for his thrombotic storm remains uncertain, anti-complement therapy with eculizumab provided rapid and durable lesion resolution. Eculizumab was discontinued after 6 months and patient remains in remission without recurrent thrombosis. This case provides insight on the management of CAPS, including the use of eculizumab.

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology.

BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called "thrombotic storm" and multiple organ failure, that is, CAPS. MATERIALS AND METHODS: Since 2001-2016, we discovered 17 patients with CAPS development. CONCLUSION: CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%-40%.

An unusual case of thrombotic storm in an amateur cricketer-a case report.

BACKGROUND: Thrombotic storm is a series of acute to subacute thrombotic events that evolve over a few days to weeks and result in progressive thromboses at multiple sites. There is often a predisposing event to thromboses, such as trauma or infections. Prompt initiation of anti-coagulation can prove life-saving in such patients. CASE REPORT: We describe a previously healthy young male who developed thromboses of the right axillary, brachial, radial, and ulnar arteries while bowling in a cricket match. A few hours later, he developed a stroke involving the right anterior and middle cerebral arteries. His thrombophilia workup was significant for elevated homocysteine levels. Although he had a delayed presentation to our hospital, he was treated with anticoagulation and given a trial of thromboembolectomy, which failed and he had to ultimately undergo a right below-elbow guillotine amputation. CONCLUSION: Thrombotic storm should be recognized promptly in the Emergency Department and timely anticoagulation should be initiated.

Thrombotic Storm Induced by Rituximab in a Patient With Pemphigus Vulgaris.

Thrombotic storm is a rare hypercoagulable condition characterized by a clinical trigger causing extensive thrombotic events affecting multiple vessels over a short period of time. We present a case of thrombotic storm that developed in a patient who received rituximab therapy. The patient presented to the hospital with dyspnea and shortness of breath and was subsequently diagnosed with extensive thrombotic burden including multiple deep vein thrombi and pulmonary emboli. Hypercoagulable workup for the thrombotic storm was unrevealing with the only identifiable trigger being the rituximab infusion. The patient was treated successfully with anticoagulation and discontinuation of rituximab. There are very few reports highlighting thrombotic events as a complication of rituximab therapy. We aim to increase recognition of thrombotic storm as a potential complication of receiving rituximab therapy.

Risk Factors, Clinical Presentation, Therapeutic Trends, and Outcomes in Arterial Thrombosis Complicating Unvaccinated COVID-19 Patients: A Systematic Review.

Data on characteristics and outcomes of coronavirus (COVID)-19 patients complicated with arterial thrombosis (AT) are scarce. Therefore, we carried out a systematic review (PRISMA, PROSPERO statements; PubMed, Scopus, and Web of Science) to identify risk factors, clinical presentation, treatment, and outcomes. We included publications from December 2019 to October 2020. Groups: (a) ischemic stroke, (b) thrombotic storm, (c) peripheral vascular thrombosis, (d) myocardial infarction, and (e) left cardiac thrombus or in-transit thrombus (venous system thrombus floating or attaching to the right heart). We considered 131 studies. The most frequent cardiovascular risk factors were: hypertension, diabetes, and dyslipidemia. A high proportion presented with asymptomatic, mild, or moderate COVID-19 (n = 91, 41.4%). We identified a high percentage of isolated ischemic stroke and thrombotic storm. Groups with higher mortality rate: intracardiac thrombus (1/2, 50.0%), thrombotic storm (18/49, 36.7%), and ischemic stroke (48/131, 36.6%). A small number received thromboprophylaxis. Most patients received antithrombotic treatment. The most frequent bleeding complication was intracranial hemorrhage, primarily with isolated stroke. Overall mortality was 33.6% (74/220). Despite a wide range of COVID-19 severity, a high proportion had AT as a complication of non-severe disease. AT can affect different vascular territories; mortality is associated with stroke, intensive care unit stay, and severe COVID-19.

A Probable Catastrophic Antiphospholipid Antibody Syndrome/Thrombotic Storm Presenting As Rapidly Evolving Multifocal Ischemic and Hemorrhagic Strokes: A Case Report.

Catastrophic antiphospholipid antibody syndrome (CAPS) is a life-threatening disorder. It is a rare and severe form of antiphospholipid antibody (APL) syndrome characterized by widespread multisystemic thrombosis. We present a 55-year-old male patient with acute cerebellar hemorrhagic stroke who developed widespread progressive microthrombosis and macrothrombosis manifesting as progressive bilateral ischemic strokes with lower extremities deep vein thrombosis (DVT) and acute renal failure within a week of presentation. The diagnosis and initiation of therapy were established after serological confirmation. This case adds to a limited number of cases of CAPS in literature and is interesting given the rarity of CAPS and thrombotic storm (TS) as well as the lack of inciting factor triggering CAPS/thrombotic syndrome. This case also helps to remind the clinicians of the importance to consider CAPS, even prior to serological confirmation, in those with rapidly progressive thrombotic events, as delayed diagnosis and therapy can yield poor clinical outcomes.

COVID-19 Infection: A Sparked Ignition to a Thrombotic Storm.

Thrombotic storm (TS) is a rare yet life-threatening condition that requires aggressive thrombolytic or anticoagulant therapy. Clinical manifestation of TS can be disastrous as it amplifies thrombotic pathways causing widespread organ ischemia. We present a patient who developed TS following a COVID-19 infection. He was simultaneously diagnosed with an ST-elevation myocardial infarction, multiple pulmonary emboli, aortic thrombi, and bilateral limb ischemia. Further workup was positive for a spindle cell neoplasm, which combined with the prothrombotic nature of COVID-19 infection likely produced an exaggerated response leading to a diffuse thrombotic event. Through this case, we would like to highlight the importance of having a collective field of expertise in making the most appropriate medical decision under critical situations.

Catastrophic antiphospholipid syndrome and pregnancy. Clinical report.

We have observed the development of a catastrophic antiphospholipid syndrome (CAPS) in a pregnant woman hospitalized at 28 weeks of gestation with a severe preeclampsia. On the same day, an eclampsia attack developed, and an emergency surgical delivery was performed. On the third day, multiorgan failure developed. Examination showed a persistent circulation of lupus anticoagulant, high level of antibodies to cardiolipin, b2-glycoprotein I, and prothrombin. The usual diagnosis of the severe preeclampsia masked a catastrophic antiphospholipid syndrome, exacerbated by the coincident presence of several types of antiphospholipid antibodies. The first pregnancy resulted in a premature birth at 25 weeks, possibly also due to the circulation of antiphospholipid antibodies. The trigger of the catastrophic form development was the pregnancy itself, surgical intervention, and hyperhomocysteinemia. CAPS is the most severe form of antiphospholipid syndrome, manifested in multiple microthrombosis of microcirculation of vital organs and in the development of multiorgan failure against the background of the high level of antiphospholipid antibodies. CAPS is characterized by renal, cerebral, gastrointestinal, adrenal, ovarian, skin, and other forms of microthrombosis. Thrombosis recurrence is typical. Thrombotic microvasculopathy lies at the heart of multiorgan failure and manifests clinically in central nervous system lesions, adrenal insufficiency, and ARDS development. CAPS is a life-threatening condition, therefore, requires an urgent treatment. Optimal treatment of CAPS is not developed. CAPS represent a general medical multidisciplinary problem.

Thrombotic storm in a 4-year-old boy with a thrombus in the right atrium.

Thrombotic storm (TS) is a rare disease, especially with thrombus in the heart of pediatric patient. We present a case of a 4-year-old boy, who was diagnosed with TS during his first hospitalization due to lower extremity deep venous thrombosis, pulmonary embolism, and thrombosis of the inferior vena cava, cerebral, left internal jugular, portal, renal, and iliac veins. He was eventually prescribed with rivaroxaban to control thrombosis after 30 days of successive use of low-molecular-weight heparin, unfractionated heparin, and warfarin, which were demonstrating little effect on preventing thrombosis, and the patient was intolerant to argatroban. While his lupus anticoagulant ratio was slightly above the normal range and no other potential causes such as congenital thrombophilia, severe infection, malignancy, and trauma were confirmed, we suspected antiphospholipid antibody syndrome and prescribed glucocorticoid and rituximab to control the disease. After 36 days of admission, ultrasonography showed recanalization of the former thrombus. One month after discharge, a tumor embolus resembling a mass emerged in his right atrium under effective anticoagulant therapy. During his second admission, he underwent surgical thrombectomy, and pathological examination confirmed the mass to be a platelet-rich thrombus rather than tumor embolus or infection. Considering the suspected antiphospholipid antibody syndrome as the cause of the TS, we prescribed aspirin combined with rivaroxaban to prevent thrombosis. In this case, surgery and pathology shed light on the type of thrombus that emerged from the inferior vena cava and traveled to the heart, which is the possible potential cause of TS. It also changed our therapeutic strategy to antiplatelet therapy combined with anticoagulant therapy to control the disease.

Kimura's disease: An uncommon cause of lymphadenopathy.

Lymph node enlargement of neck and axilla is one of the common presenting complaints in pediatrics. We are presenting here a very rare cause of axillary lymphadenopathy detected in a toddler.