1/13. Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/13. A role for transoesophageal echocardiography in the early diagnosis of catastrophic antiphospholipid syndrome.We describe a previously healthy 28-year-old woman who presented with the clinical picture of large vessel occlusions (stroke with left hemiparesis, myocardial infarction) and developed multi-organ failure (i.e. kidneys, heart, brain, liver, blood, skin) over a very short period of time. Peripheral blood smear was consistent with thrombotic thrombocytopenic purpura. Transesophageal echocardiogram was supportive of the diagnosis of catastrophic antiphospholipid syndrome (CAPS), revealing Libman-Sacks endocarditis. Blood cultures were negative, anticardiolipin antibodies were highly increased and lupus anticoagulant was positive. Cerebral and coronary angiograms were negative, suggesting possible microthrombotic occlusive disease in the setting of CAPS.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
3/13. Fatal malignant hyperthermia--delayed onset and atypical course.A case of malignant hyperthermia (mh) in a 27-year-old man is described. In a first anaesthesia using isoflurane and succinylcholine, the end-tidal CO(2) rose from 39 to 49 mmHg 2.75 h post-intubation and the body temperature rose to 39.8 degrees C 14 h post-intubation but was normal again the next day. In a second anaesthesia using the same medication, the maximal end-tidal CO(2) was 44 mmHg and the body temperature rose to 39 degrees C after 9 h. After 4 days, the fever rose to 40 degrees C, and to 42 degrees C when death occurred 10 days after the second anaesthesia. Masseter spasms or muscle rigidity were never present. According to the death certificate, death was due to multi-organ failure from sepsis. At autopsy, the skeletal muscles were pale and oedematous. histology demonstrated focal necroses in the skeletal muscles, shock kidneys with myoglobin excretion and myoglobin clots in small blood vessels of the lungs. Hence, the postmortem diagnosis "malignant hyperthermia" was established but accusations of medical maltreatment were rejected because of the atypical and protracted clinical course and because uncharacteristic signs of malignant hyperthermia were attributable to the clinically suspected sepsis.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
4/13. Primary antiphospholipid syndrome with multiorgan arterial and venous thromboses.A 57-year-old man with no evidence of infection, vasculitis or connective tissue disease died with multiple organ thromboses after an acute illness. He was found to have lupus anticoagulant, IgG anticardiolipin antibody, false positive rapid plasma reagin, prolonged partial thromboplastin time, and thrombocytopenia. Venous and arterial thrombi leading to necrosis were found in his scrotum, testicles, upper and lower extremities, adrenals, kidneys, lungs, and brain. No other explanation could be found for his fatal illness, thus suggesting the primary antiphospholipid syndrome (APS). This is a documented case of primary APS associated with multiorgan arterial and venous thromboses of large and small vessels, presenting as a fulminant and fatal acute illness.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
5/13. A case of intravascular large B-cell lymphoma with multiple organ involvement.Intravascular large B-cell lymphoma (IVL) is a rare systemic disease characterized by clonal proliferation of neoplastic lymphoid cells within the capillary lumina of small blood vessels. The most common sites of involvement are cerebrovascular or sinusoid areas, resulting in a wide variety of neurologic deficits. Herein we describe a case of IVL that presented with multiple internal and external organ involvements, including the skin, bilateral kidneys, and lung, and with an aggressive clinical course. The confirmative diagnosis was based on the microscopic findings of the skin lesion with a B-cell immunophenotype. The patient suffered from repeated severe dyspnea, hypoxemia, and high-grade fever with a simultaneous increase in serum lactate dehydrogenase and soluble IL-2 receptor levels. These manifestations responded dramatically to intravenous injection of corticosteroid (500 mg/day for 3 days). Despite various clinical presentations, no neurological abnormality was observed until the patient died 12 months after the initial combination chemotherapy.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
6/13. Recurrent gastric hemorrhaging with large submucosal hematomas in a patient with primary AL systemic amyloidosis: endoscopic and histopathological findings.A 64-year-old woman who suffered intractable gastric ulcers with hemorrhaging showed huge submucosal hematomas in her stomach on the endoscopic examination. Since gastric mucosal biopsy revealed amyloid deposition and IgG lambda type M protein was detectable in her serum, she was diagnosed as having primary AL systemic amyloidosis. The gastric hemorrhages did not improve despite intensive medication, so total gastrectomy was performed, resulting in an unfavorable outcome. Massive deposition of amyloid with A lambda immunoreactivity was seen on the submucosal vessels in her stomach. This is a rare primary AL systemic amyloidosis case showing recurrent and fatal gastric submucosal hematomas.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
7/13. Intravascular lymphoma - a rare cause of hemolytic anemia and neurologic disorders.Intravascular lymphoma is an uncommon and often overlooked form of non-Hodgkin's lymphoma characterized by extensive proliferation of lymphoid cells within the lumina of small and medium-sized vessels. Clinical symptoms of the disease are variable and often nonspecific, mostly neurologic in nature. With an aggressive course, intravascular lymphomatosis has a poor prognosis and is rarely diagnosed ante mortem. We describe here a 76-year-old woman with the clinical diagnoses of hemolytic anemia and progressive lethargy where intravascular lymphomatosis turned out as the underlying cause of the disease.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
8/13. heparin-induced thrombocytopenia after coronary artery bypass grafting with cardiopulmonary bypass: report of a case.A 79-year-old man was transferred to our hospital with severe chest pain and a suspected diagnosis of acute myocardial infarction. Emergency cardiac catheterization showed triple-vessel coronary artery disease, and we performed coronary artery bypass grafting under cardiopulmonary bypass (CPB). Continuous hemodiafiltration was started for acute renal failure postoperatively, and heparin was given as an anticoagulanting agent. By 9 days after the initiation of heparin therapy, his platelet count had fallen and a deep vein thrombosis had formed in his left leg. We suspected heparin-induced thrombocytopenia (HIT), and immediately discontinued the heparin, implementing danaparoid (Orgaran) instead, following which the platelet count recovered. heparin-induced thrombocytopenia, which causes thrombosis, is a serious side effect of heparin therapy and few cases of HIT associated with CPB surgery have been reported in japan.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
9/13. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
10/13. Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: case report.Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high-grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifestations make antemortem diagnosis very difficult. We report herein a case of IVL revealed by fatal, precapillary, pulmonary arterial hypertension and associated with long-lasting fever. Extensive investigation of the usual causes of pulmonary arterial hypertension was negative. The diagnosis of intravascular lymphoma was made on postmortem analysis, revealing diffuse and sometimes complete obliterations of the lumina of small blood vessels by large B-cell lymphoma, including pulmonary capillaries. Thus, we propose that IVL must be added to the spectrum of etiologies of subacute pulmonary arterial hypertension, notably in the context of associated fever, both entities requiring emergency diagnosis. For this purpose, blood collected via pulmonary capillary-wedge aspiration for cytologic examination may be associated with right-sided heart catheterization when this latter procedure is suitable.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
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