Cases reported "Catastrophic Illness"

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1/4. Catastrophic arterial reactivity during primary antiphospholipid syndrome--a case report.

    Arterial reactivity leading to acute thrombosis at the site of a needle stick injury has never been described during antiphospholipid syndrome. The authors report a case characterized by a succession of thrombotic events occurring during or immediately after arterial angiographies or arterial surgery, in which catastrophic arterial reactivity can be strongly suspected. In this particular patient, it can be postulated that damage to the endothelial cells of the vessels injured during manipulation may have precipitated or aggravated the preexisting susceptibility to thrombosis.
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2/4. Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury - a case report with pathological and molecular correlative studies.

    Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare variant of antiphospholipid syndrome (APS) characterized by acute multiorgan failure due to small vessel thrombi in patients with positive antiphospholipid antibodies. We report a fatal case of catastrophic antiphospholipid syndrome in a young woman with a history of polymyositis and Hodgkin lymphoma. The patient was admitted to hospital because of severe foot pain following several weeks of skin ulcerations. Doppler ultrasonography showed evidence of arterial ischemia of the both lower extremities. Despite anticoagulation, immunosuppression, plasmapheresis and antibiotic therapy, she developed cutaneous gangrene, retroperitoneal hematoma, ileus, and acute respiratory and renal failure that resulted in death. autopsy showed multifocal vascular injury and microthrombi with associated hemorrhages and infarcts in multiple organs. The patient had normal levels of functional protein c and protein s and a normal level of plasma homocysteine. Tests for common thromophilic gene mutations including prothrombin 20210, factor v Leiden 1691, and methylene tetrahydrofolate reductase 677 were negative. To our knowledge, this is the first CAPS patient with molecular studies for genetic prothrombotic mutations. Our report showed that there was no association between the development of CAPS and inherited thromophilia.
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3/4. Catastrophic antiphospholipid antibody syndrome.

    Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.
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4/4. Catastrophic thromboses and severe thrombocytopenia during heparin therapy in a patient with anti-phospholipid syndrome.

    Catastrophic anti-phospholipid syndrome (CAPS) is a medical emergency characterized by thromboses of multiple small vessels of internal organs and the brain. Herein we present a patient with primary anti-phospholipid syndrome who developed CAPS manifested by hepatic, renal and splenic artery thromboses, as well as cerebral venous thrombosis. The course was further complicated by severe thrombocytopenia and haemolytic anemia. Two episodes of catastrophic thrombosis developed within 24-36 h after the initiation of heparin therapy, suggesting a role of heparin in triggering thromboses. The patient had no anti-platelet-factor-4 antibodies in repeated measurements, making clinical diagnosis of heparin-induced thrombocytopenia unlikely. The possible role of heparin in induction of thromboses and its therapeutic implication are detailed.
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