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1/17. 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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2/17. Anti-thymocyte globulin treatment of marrow aplasia associated with paroxysmal nocturnal haemoglobinuria (PNH) resulted in haematological improvement due to an expansion of the PNH clone.

    A patient with aplastic anaemia developed paroxysmal nocturnal haemoglobinuria (PNH) 4 years after diagnosis, with an ensuing haematopoietic improvement. The PNH clone subsequently declined, leading to pancytopenia again. Anti-thymocyte globulin had to be administered 14 years later, which resulted in haematopoietic improvement once more. Flow cytometric analysis showed that this was attributable to expansion of the PNH clone, owing probably to alleviation of its suppression by immune-mediated mechanisms. PIG-A gene analysis showed that the same PNH clone had waned and waxed in the clinical course. Our results suggest that the PNH clone might rarely be an immune target as well.
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3/17. 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.
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4/17. Off-pump coronary artery bypass grafting in a patient with paroxysmal nocturnal hemoglobinuria.

    Paroxysmal nocturnal hemoglobinuria has not been described in patients undergoing off-pump coronary artery bypass grafting. A 65-year-old man who underwent percutaneous coronary stenting to the proximal left anterior descending artery for unstable angina was readmitted to our hospital complaining of recurrent chest pains. A coronary angiography revealed in-stent restenosis and new lesions of the distal left anterior descending artery as well as the left circumflex artery branch. He was found to have paroxysmal nocturnal hemoglobinuria which contributes to serious surgical complications including infection, bleeding, hemolysis and acute renal failure. After pancytopenia was treated with administration of granulocyte colony stimulating factor and transfusion of the washed red blood cells preoperatively, off-pump coronary artery bypass grafting was performed. cardiopulmonary bypass was avoided in order to reduce activation of complements. His postoperative course was uneventful. Combination of appropriate perioperative management and off-pump cardiac surgery yielded an effective result in treating this patient without major complications.
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5/17. Late marrow allograft rejection following alpha-interferon therapy for hepatitis in a patient with paroxysmal nocturnal hemoglobinuria.

    We describe a case of allograft rejection that occurred 23 months after successful bone marrow transplantation for severe aplastic anemia in a patient with paroxysmal nocturnal hemoglobinuria. The allograft rejection appears to have been induced by recombinant alpha-interferon (rINF-alpha) treatment for non-A, non-B hepatitis that developed 11 months after transplantation. During the 9 months of active hepatitis, the donor graft functioned normally; however, 3 months after rINF-alpha therapy was started, pancytopenia and a chimeric hematopoietic state developed. rINF-alpha was discontinued, cyclosporin A was reintroduced, and autologous bone marrow recovery followed. rINF-alpha treatment may be detrimental to some recipients of allogeneic bone marrow transplants.
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6/17. Paroxysmal nocturnal hemoglobinuria with onset in childhood: a case report.

    A twelve-year-old boy presented with recurrent episodes of anemia. Complete blood counts showed pancytopenia. Bone marrow was hypercellular with erythroid hyperplasia and depleted stores of iron. Positive Ham's test and sucrose lysis test revealed that he had paroxysmal nocturnal hemoglobinuria. There was a delay of nearly two years in the diagnosis in this patient. Paroxysmal nocturnal hemoglobinuria is rare in childhood. It must however be considered in a child who presents with unexplained anemia or bone marrow failure so that an early and accurate diagnosis is reached.
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7/17. Paroxysmal nocturnal hemoglobinuria in childhood: an uncommon presentation.

    An eight year old boy presented with severe anemia and bleeding spots. Complete blood count showed pancytopenia. There was mild reticulocytosis. Bone marrow was hypocellular with normoblastic erythroid hyperplasia. Ham's test (acidified serum test) was positive which confirmed the diagnosis of Paroxysmal nocturnal hemoglobinuria (PNH). Although PNH is rare in childhood, it should be considered as a diagnostic possibility in cases of aplastic anemia as the two conditions can coexist. The presence of PNH in association with aplastic anemia can influence the outcome of the latter.
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8/17. Placental expression of glycophosphatidylinositol (GPI)-anchored proteins in paroxysmal nocturnal haemoglobinuria.

    Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal stem cell disorder in which a defect of glycophosphatidylinositol (GPI)-anchored proteins leads to higher morbidity and mortality because of intravascular haemolysis, haemoglobinuria, pancytopenia and an increased frequency of thrombotic events. We report here the clinical features of a pregnant woman with PNH and present an immunhistochemical analysis of complement regulators, leukocyte activation markers and placental alkaline phosphatase (PALP) on syncytiotrophoblasts and inflammatory cells in her placenta. Placental tissue from normal deliveries served as controls. The patient had severe PNH with haemolysis, thrombosis episodes and signs of bone marrow failure. Placental syncytiotrophoblasts and villous cells of fetal origin in both normal placentas and the placenta from the PNH patient expressed PALP and the complement regulators CD46, CD55 and CD59. Additionally, CD11b-positive leukocytes of presumed maternal origin were negative for CD15 in the PNH placenta, while they stained positive within the villous space and in normal placentas. These findings show that fetally derived cells in the PNH placenta expressed GPI-linked molecules that are known to be of importance for a successful pregnancy outcome.
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9/17. Paroxysmal nocturnal hemoglobinuria: report of one case.

    Paroxysmal nocturnal hemoglobinuria (PNH) is a complex stem cell disorder and its occurrence in childhood is quite uncommon. A 6-year-old girl with pancytopenia was presented. There is no nocturnal hemoglobinuria or other symptoms of chronic hemolysis. bone marrow examination revealed mild hypocellularity initially, and a tentative diagnosis of aplastic anemia was made. This patient received conventional therapy with uneventful course. Two consecutive episodes of hemolytic transfusion reaction were noted and positive sugar water test and Ham,s test lead the clue of PNH. The literature on the clinical manifestation, pathogenesis, diagnosis and management of PNH is reviewed briefly.
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10/17. Transient pancytopenia associated with parvovirus infection in paroxysmal nocturnal haemoglobinuria.

    A 25 year old woman with a 15-year history of paroxysmal nocturnal haemoglobinuria developed transient pancytopenia following infection with human parvovirus B19. This is the first report of transient pancytopenia in a patient with an acquired haemolytic anaemia due to parvovirus. The possible mechanism of pancytopenia in such a case is discussed.
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