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1/62. portal vein thrombosis in paroxysmal nocturnal haemoglobinuria.

    A 28-year-old man was hospitalized with nausea, vomiting, abdominal pain and low-grade fever. He had a 6-month history of paroxysmal nocturnal haemoglobinuria (PNH), and laboratory data showed anaemia and liver dysfunction. An abdominal ultrasonography showed ascites and portal vein thrombosis. After receiving antithrombotic treatment, the portal vein thrombosis did not extend. portal vein thrombosis is very rare but should be considered when we encounter liver dysfunction associated with PNH as well as hepatic vein thrombosis. ultrasonography is very useful in detecting portal vein thrombosis and facilitating early diagnosis. warfarin is very effective in preventing exacerbation of portal vein thrombosis in PNH.
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ranking = 1
keywords = thrombosis, vein thrombosis, vein
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2/62. The anesthetic management of a patient with paroxysmal nocturnal hemoglobinuria.

    Implications: This case report describes the anesthetic considerations for a patient with paroxysmal nocturnal hemoglobinuria. Specific strategies to be applied in the perioperative period to prevent hemolytic episodes and venous thrombosis are also discussed.
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ranking = 0.12345010240202
keywords = thrombosis, venous thrombosis
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3/62. Reversible renal failure in paroxysmal nocturnal hemoglobinuria.

    A Chinese patient with paroxysmal nocturnal hemoglobinuria (PNH) developed acute nonoliguric renal failure with intercurrent urinary tract infection and hemolysis. There was no evidence of renal vein thrombosis. Renal biopsy showed features of acute tubular necrosis (ATN) and hemosiderosis. magnetic resonance imaging (MRI) showed characteristic features of renal hemosiderosis. The patient was stabilized with temporary hemodialysis and intravenous fluid. The renal function fully recovered 3 weeks later. We review the literature and summarize the clinical features of this disease entity. To our knowledge, this case is the first to report such disorder with thorough investigation including concomitant diagnostic MRI imaging and renal biopsy.
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ranking = 0.10063070275326
keywords = thrombosis, vein thrombosis, vein
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4/62. Successful application of nonmyeloablative transplantation for paroxysmal nocturnal hemoglobinuria.

    OBJECTIVE: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder that manifests as hemolytic anemia, venous thrombosis, and deficient hematopoiesis. Although allogeneic hematopoietic stem cell transplantation is considered the only curative therapeutic measure, transplant-related mortality is not negligible. Several studies supported the use of nonmyeloablative stem cell transplantation (NST) for patients of advanced age or with organ dysfunction. Hence, we used NST in a PNH patient who suffered from acute renal failure due to repeated episodes of hemolysis. MATERIALS AND methods: We performed NST using a conditioning regimen consisting of cladribine 0.11 mg/kg x 6, busulfan 4 mg/kg x 2, and rabbit anti-thymocyte globulin 2.5 mg/kg x 2. He received peripheral blood stem cells from his human leukocyte antigen-matched brother. Prophylaxis against graft-vs-host disease was performed with cyclosporine A alone. chimerism of peripheral blood mononuclear cells was evaluated serially using short tandem repeat analysis and flow cytometry. RESULTS: No meaningful regimen-related toxicities were documented. Donor chimerism of 90 to 100% was achieved on day 14 and thereafter. The patient is doing well, without any recurrence of hemolysis 6 months after transplant. Follow-up chimerism studies confirmed stable and functioning donor-type hematopoiesis. CONCLUSIONS: NST may become a safe and curative approach in patients with PNH. Further studies are needed to establish the role of NST for treatment of PNH.
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ranking = 0.12345010240202
keywords = thrombosis, venous thrombosis
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5/62. Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT.

    Paroxysmal nocturnal hemoglobinuria is a rare disorder characterized by pancytopenia. One of the main manifestations of this disease is development of widespread life-threatening venous thrombosis, which may involve multiple abdominal veins. We describe two patients with paroxysmal nocturnal hemoglobinuria with clinically unsuspected portal, splenic, and mesenteric venous thromboses that were diagnosed on computed tomography. This complication should be clinically suspected in patients suffering from paroxysmal nocturnal hemoglobinuria who present with abdominal pain, and it should be sought by the radiologist on pre- and postcontrast computed tomography.
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ranking = 0.61750387939993
keywords = thrombosis, venous thrombosis, vein
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6/62. Acute renal failure in a patient with paroxysmal nocturnal hemoglobinuria.

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by low-grade, chronic hemolytic anemia accompanied by either thrombocytopenia or leucopenia. kidney involvement is usually benign and secondary to chronic tubular deposition of hemosiderin. Acute renal failure may occur in association with a hemolytic crisis. We report the case of a 70-year-old Caucasian woman with PNH who developed reversible acute renal failure requiring hemodialysis following a gastointestinal illness. Renal biopsy demonstrated acute tubular necrosis with considerable hemosiderin deposition, but no evidence of vascular thrombosis.
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ranking = 0.094070307830794
keywords = thrombosis
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7/62. Cerebral venous thrombosis in a patient of paroxysmal nocturnal haemoglobinuria following aplastic anaemia.

    A young lady who had aplastic anaemia presented for cerebral venous thrombosis after five years of follow up. She was diagnosed to have paroxysmal nocturnal haemoglobinuria. She had received immunosuppressive therapy with methylprednisolone, cyclosporine-A, anti-lymphocyte globulin, danazol and pregnenolone. The relation between aplastic anaemia, paroxysmal nocturnal haemoglobinuria and cerebral venous thrombosis is discussed. The role of immunosuppressive therapy for aplastic anaemia in causation of paroxysmal nocturnal haemoglobinuria is reviewed.
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ranking = 0.74070061441213
keywords = thrombosis, venous thrombosis
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8/62. Gastrointestinal involvement in paroxysmal nocturnal hemoglobinuria: first report of electron microscopic findings.

    Thrombotic complications, particularly microthrombi involving intraabdominal veins leading to intestinal ischemia, have remained a major cause of morbidity in patients with paroxysmal nocturnal hemoglobinuria (PNH). While intestinal ischemia has been postulated to be the cause of recurrent bouts of abdominal pain in this population, direct antemortem evidence for this complication is scarcely documented in the literature. We describe a case of PNH in a patient who presented with abdominal distress three years after the initial diagnosis was established. Clinical features and a combination of diagnostic modalities, including radiography, endoscopy, and histology were used to make the prompt diagnosis of intestinal ischemia. This is the first case in which the electronic microscopy of the gastrointestinal lesion is described. Our patient was successfully treated with conservative measures and anticoagulation.
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ranking = 0.00025336738982507
keywords = vein
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9/62. Platelet hypersensitivity and intravascular coagulation in paroxysmal nocturnal hemoglobinuria.

    The patient described had paroxysmal nocturnal hemoglobinuria associated with recurrent arterial as well as venous thrombosis. Study of platelet function revealed hypersensitivity to epinephrine, adenosine 5'phosphate (ADP) and collagen as judged by their ability to aggregate platelets as well as to release 14C serotonin. The release of total nucleotides was also markedly increased over normal with all aggregating agents. The abnormality was localized to the platelet since aggregation occurred when the patient's platelets were resuspended in normal plasma but not when normal platelets were incubated in the patient's plasma. Presumptive evidence for ongoing intravascular coagulation was an increase in fibrinogen derivatives of heavier molecular weight than the native protein presumably a result of thrombin action. However, factor xii was not activated and fibrinolysis was not increased. Complement component levels and antithrombin concentrations were also normal. The findings in this case suggest that hypersensitive platelets may contribute to the intravascular coagulation that is manifested by the increased incidence of thrombosis in patients with paroxysmal nocturnal hemoglobinuria.
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ranking = 0.21752041023282
keywords = thrombosis, venous thrombosis
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10/62. Stem cell transplantation for paroxysmal nocturnal haemoglobinuria in childhood.

    Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal haematopoietic disorder characterized by chronic or intermittent intravascular haemolysis, variable cytopenia and an increased risk of thrombosis. Stem cell transplantation (SCT) is a curative therapeutic option, but its risks must be carefully weighed against the natural course of PNH. World-wide experience with SCT for PNH in the paediatric age group is scarce. We report on two adolescents suffering from PNH with life-threatening complications who were successfully transplanted from unrelated donors. Indications and techniques of SCT in childhood PNH are discussed and an overview of the literature is given.
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ranking = 0.094070307830794
keywords = thrombosis
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