1/6. Adult Henoch-Schonlein purpura with glomerulonephritis and paroxysmal nocturnal haemoglobinuria: an uncommon association.Henoch-Schonlein purpura (HSP) is considered to be a small blood vessel systemic vasculitis. Numerous microorganisms have been implicated in triggering HSP. We describe an unusual case of HSP with glomerulonephritis and renal failure requiring haemodialysis in a young adult man who subsequently developed paroxysmal nocturnal haemoglobinuria (PNH) with several haemolytic episodes. bacterial infections, especially those of the respiratory and urinary tract, might trigger both the diseases.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/6. splenectomy for massive splenic infarction unmasks paroxysmal nocturnal hemoglobinuria.Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by pancytopenia, hemolysis, and thrombosis. Abdominal vein thrombosis is a life-threatening manifestation of this disease. We present a patient with complete spleen necrosis due to thrombosis of the splenic vessels. After splenectomy, other causes of thrombophilia were excluded and the diagnosis of PNH was established. The patient was put on anticoagulation but despite the prophylactic international normalized ratio maintained over the last 18 months of follow-up, he had another episode of intrahepatic thrombosis which was treated with tissue plasminogen activator thrombolysis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/6. Catheter-directed thrombolysis and thrombectomy for the budd-chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. patients with hepatic vein thrombosis (budd-chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/6. Paroxysmal nocturnal haemoglobinuria and budd-chiari syndrome.An 11 year old boy developed pancytopenia, haemolysis, and budd-chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/6. Morphological picture in paroxysmal nocturnal hemoglobinuria. Case report.25-year-old woman with paroxysmal nocturnal hemoglobinuria was admitted to the hospital because of headache, progressing right hemiparesis and speech disorders. Several days later patient lost consciousness. cerebrospinal fluid was xanthochromic with increased pleocytosis and protein level. CT-scan revealed ischemic area with hemorrhagic focus within left cerebral hemisphere. Patient died 3 weeks after the admission. brain section revealed hemorrhagic infarct in the cortex of the left parietal lobe, thrombosis of the superior sagittal sinus and "respiratory brain" changes. Microscopic examination revealed meningeal venous thrombosis, hemorrhagic infarct, vasculitis, abundant accumulation of bacteria within blood vessels, and other pathological changes such as petechiae, perivascular exudates and small, round areas composed of acellular fibrillary network. There were no macrophages and GFAP-positive astrocytes in any of these areas. Authors suggest that weak cell reactivity may be connected with alterations in cell membranes, mainly low phosphatidylinositol (GPI) content.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/6. Paroxysmal nocturnal hemoglobinuria presenting as moyamoya syndrome.We report an 11-year-old girl who has paroxysmal nocturnal hemoglobinuria (PNH) and was admitted because of recurrent cerebrovascular accidents (CVA) and intermittent hemoglobinuria. Internal carotid angiography revealed bilateral typical moyamoya patterns. Although CVA due to arterial thrombosis may occur in PNH, the basal moyamoya vessels were never mentioned in case reports yet. The moyamoya syndrome has been reported in a variety of diseases and represents the nonspecific response to an impairment of arterial flow at specific sites in the brain. Our case discloses that PNH may present as moyamoya syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |