|1.||Venous Thrombosis (Deep-Vein Thrombosis)
|4.||Aplastic Anemia (Anemia, Hypoplastic)
|5.||Myelodysplastic Syndromes (Myelodysplastic Syndrome)
|1.||Brodsky, Robert A: 25 articles (12/2015 - 04/2004)|
|2.||Risitano, Antonio M: 23 articles (12/2015 - 05/2002)|
|3.||Hillmen, Peter: 20 articles (05/2014 - 05/2002)|
|4.||Luzzatto, Lucio: 17 articles (02/2014 - 09/2002)|
|5.||Young, Neal S: 16 articles (09/2014 - 05/2002)|
|6.||Hill, Anita: 15 articles (09/2015 - 10/2005)|
|7.||Notaro, Rosario: 13 articles (03/2014 - 09/2002)|
|8.||Schrezenmeier, Hubert: 12 articles (09/2015 - 04/2003)|
|9.||Kinoshita, Taroh: 12 articles (02/2014 - 02/2002)|
|10.||Rother, Russell P: 12 articles (08/2010 - 02/2004)|
06/23/2011 - "Long-term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survival."
04/01/2012 - "The research discussed in this review describes development of eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and the efficacy of eculizumab on allergen-induced asthmatic responses in a placebo-controlled study. "
01/01/2013 - "Efficacy of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria requiring transfusions 14 years after a diagnosis in childhood."
10/04/2006 - "[Evidence of the efficacy of eculizumab in paroxysmal nocturnal hemoglobinuria]."
09/23/2015 - "Long Standing Eculizumab Treatment without Anticoagulant Therapy in High-Risk Thrombogenic Paroxysmal Nocturnal Hemoglobinuria."
|2.||Complement System Proteins (Complement)IBA
05/04/2012 - "A novel therapeutic reagent TT30 was designed to be effective in diseases of the alternative pathway of complement such as paroxysmal nocturnal hemoglobinuria and other diseases. "
04/01/1965 - "THE INITIATION AND ENHANCEMENT OF HUMAN RED CELL LYSIS BY ACTIVATORS OF THE FIRST COMPONENT OF COMPLEMENT AND BY FIRST COMPONENT ESTERASE; STUDIES USING NORMAL RED CELLS AND RED CELLS FROM PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA."
09/01/2015 - "No established therapy exists for secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria, and the possibility of therapeutic complement inhibition is interesting. "
09/01/2015 - "Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. "
08/13/2015 - "Complement C3dg-mediated erythrophagocytosis: implications for paroxysmal nocturnal hemoglobinuria."
|3.||Granulocyte Colony-Stimulating Factor (G-CSF)IBA
10/01/1997 - "Paroxysmal nocturnal hemoglobinuria: efficacy of prolonged treatment with granulocyte colony-stimulating factor."
04/10/2012 - "To study the expressions of phosphorylated STAT5 (P-STAT5) in CD34(+)CD59(-) and CD34(+)CD59(+) bone marrow cells of the patients with paroxysmal nocturnal hemoglobinuria (PNH) before and after in vitro G-CSF or SCF stimulation, then evaluate the functions of G-CSF and SCF receptors in PNH clone cells. "
04/01/2005 - "To study the response of hematopoietic cells (HSC) to granulocyte colony stimulating factor (G-CSF) in paroxysmal nocturnal hemoglobinuria (PNH) patients. "
04/10/2012 - "[Expression of phosphorylated STAT5 in bone marrow hematopoietic stem cells of patients with paroxysmal nocturnal hemoglobinuria before and after in vitro G-CSF or SCF stimulation]."
04/01/2005 - "[The response of bone marrow hematopoietic cells to G-CSF in paroxysmal nocturnal hemoglobinuria patients]."
|4.||Proteins (Proteins, Gene)IBA
08/01/2011 - "Flow cytometric analysis of GPI-anchored proteins (GPI-AP) is the gold standard for diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). "
01/01/2013 - "Recently, the first of these CR2-targeted proteins has entered human phase I studies in the human disease paroxysmal nocturnal hemoglobinuria. "
01/01/2013 - "Paroxysmal nocturnal hemoglobinuria is a clonal disorder caused by acquired somatic mutations in the PIG-A gene on the X- chromosome of hemopoietic stem cells and leads to deficiency of surface membrane anchor proteins. "
01/01/2012 - "Paroxysmal nocturnal hemoglobinuria is an acquired clonal disease characterized by proliferation of stem cells, deficient of proteins linked to the membrane via glycophosphatidylinositol (GPI) anchors. "
07/01/2010 - "Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by a somatic mutation in the PIGA gene, leading to a deficiency of proteins linked to the cell membrane via glycophosphatidylinositol (GPI) anchors. "
10/01/1985 - "[Study of Donath-Landsteiner antibody in non-syphilitic paroxysmal cold hemoglobinuria]."
01/01/2000 - "[A boy with summer onset paroxysmal cold hemoglobinuria induced by Donath-Landsteiner antibody with anti-I specificity]."
01/01/1996 - "A nonspecific cold agglutinin, anti-P1, and a hemolytic biphasic Donath-Landsteiner antibody were demonstrated in his serum, and he was diagnosed with paroxysmal cold hemoglobinuria (PCH). "
07/01/1978 - "Measurement of Donath-Landsteiner antibody-producing cells in idiopathic nonsyphilitic paroxysmal cold hemoglobinuria (PCH) in children."
10/01/1971 - "Paroxysmal cold hemoglobinuria: report of a case with an exceptionally high thermal range Donath-Landsteiner antibody."
|6.||Urokinase-Type Plasminogen Activator (Urokinase)FDA Link
05/01/2002 - "Diagnostic significance of measurement of the receptor for urokinase-type plasminogen activator on granulocytes and in plasma from patients with paroxysmal nocturnal hemoglobinuria."
11/01/2001 - "To investigate the expression levels of the receptor for urokinase-type plasminogen activator (uPAR) on granulocytes and the soluble uPAR (suPAR) level in plasma from patients with paroxysmal nocturnal hemoglobinuria (PNH) and its clinical application in the diagnosis of this disorder. "
11/01/2001 - "[Diagnostic significance of measurement of the receptor for urokinase-type plasminogen activator on granulocytes and in plasma from patients with paroxysmal nocturnal hemoglobinuria]."
04/01/1997 - "Excess soluble urokinase-type plasminogen activator receptor in the plasma of patients with paroxysmal nocturnal hemoglobinuria inhibits cell-associated fibrinolytic activity."
09/01/1992 - "A soluble form of the glycolipid-anchored receptor for urokinase-type plasminogen activator is secreted from peripheral blood leukocytes from patients with paroxysmal nocturnal hemoglobinuria."
10/01/1985 - "These studies suggest that both streptokinase and urokinase are safe and effective in the treatment of intra-abdominal venous thromboses associated with paroxysmal nocturnal hemoglobinuria."
03/01/1960 - "Effect of streptokinase, epinephrine and plasmin on the paroxysmal nocturnal hemoglobinuria serum hemolytic system."
|8.||thymidine 5'-triphosphate (TTP)IBA
07/01/2014 - "These linkages may help to explain enigmatic clinical problems related to thrombotic microangiopathies, including some cases of refractory thrombotic thrombocytopenic purpura (TTP), TTP associated with only mild-modest deficiencies of ADAMTS-13, the provocation (or exacerbation) of acute episodes in patients with the atypical hemolytic uremic syndrome, and thrombosis in paroxysmal nocturnal hemoglobinuria. "
01/01/2002 - "Our model with its list of target organs similar to that observed in human diseases characterized by hemolysis and thrombosis [for example, thalassemia, sickle cell disease (SCD), paroxysmal nocturnal hemoglobinuria (PNF), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS)] suggests that it can be an excellent tool to study the pathogenesis of such complications."
|9.||Atypical hemolytic uremic syndromeIBA
02/01/2015 - "Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. "
11/02/2015 - "The annual cost of eculizumab maintenance therapy in paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic-uremic syndrome (aHUS) exceeds $300,000 per patient. "
01/01/2014 - "An orphan medicinal product, eculizumab is approved in Japan and globally for treating paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome. "
11/01/2011 - "Eculizumab, an anti-C5 monoclonal antibody, is a novel therapy of choice for patients with paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome. "
09/01/2015 - "Diseases characterized by complement hyperactivity such as paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome have high rates of thrombosis. "
|10.||Danazol (Azol)FDA LinkGeneric
12/01/2001 - "Monitoring of CD59 expression in paroxysmal nocturnal hemoglobinuria treated with danazol."
02/01/1997 - "Danazol for paroxysmal nocturnal hemoglobinuria."
09/01/1990 - "[Treatment of paroxysmal nocturnal hemoglobinuria with danazol]."
07/01/1990 - "Danazol was administered to two patients with paroxysmal nocturnal hemoglobinuria (PNH) with a dramatic effect on the hematological findings. "
07/01/1990 - "Danazol treatment for paroxysmal nocturnal hemoglobinuria."
|1.||Hematopoietic Stem Cell Transplantation
06/01/2010 - "The findings of this study confirm that most patients with paroxysmal nocturnal hemoglobinuria may be definitively cured with hematopoietic stem cell transplantation."
12/01/2015 - "The combined absence of a minor paroxysmal nocturnal hemoglobinuria population and a short telomere length is an efficient predictor of poor immunosuppressive therapy response, which should be considered while deciding treatment options: immunosuppressive therapy or first-line hematopoietic stem cell transplantation. "
06/01/2010 - "The aim of this retrospective study was to assess the long-term clinical and hematologic results in 26 paroxysmal nocturnal hemoglobinuria patients who received hematopoietic stem cell transplantation in Italy between 1988 and 2006. "
06/01/2010 - "Paroxysmal nocturnal hemoglobinuria is an acquired clonal disorder of the hemopoietic stem cells for which the only curative treatment is allogeneic hematopoietic stem cell transplantation. "
06/01/2010 - "Hematopoietic stem cell transplantation for paroxysmal nocturnal hemoglobinuria: long-term results of a retrospective study on behalf of the Gruppo Italiano Trapianto Midollo Osseo (GITMO)."
04/01/1992 - "Between 1971 and 1990, nine patients ranging in age from 14-38 years received marrow transplants for paroxysmal nocturnal hemoglobinuria (PNH). "
03/01/2008 - "The significance of detecting small populations of such paroxysmal nocturnal hemoglobinuria (PNH)-type cells was substantiated by a recent observation that PNH-type cells arose from a donor-derived hematopoietic stem cell with a PIG-A mutation in an aplastic anemia patient with late graft failure which responded well to immunosuppressive therapy. "
09/01/2008 - "Liver transplant is an appropriate treatment for Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria. "
09/01/2008 - "We report on a 20-year-old man with advanced Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria, who underwent an emergency liver transplant. "
09/01/2008 - "Liver transplant for Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria."
|3.||Transplantation (Transplant Recipients)
06/01/2015 - "[Unrelated cord blood transplantation in the treatment of paroxysmal nocturnal hemoglobinuria: a case report and literature review]."
01/01/2005 - "First two successful unrelated donor bone marrow transplantations for paroxysmal nocturnal hemoglobinuria in Poland."
05/01/2001 - "Successful application of nonmyeloablative transplantation for paroxysmal nocturnal hemoglobinuria."
04/01/1992 - "Marrow transplantation for paroxysmal nocturnal hemoglobinuria."
08/01/1984 - "Paroxysmal nocturnal hemoglobinuria apparently can be treated successfully by allogeneic or syngeneic marrow transplantation without subsequent maintenance therapy."
10/01/2011 - "[Novel therapeutics for paroxysmal nocturnal hemoglobinuria]."
03/01/2013 - "We then review the current understanding of the role of nitric oxide in sickle cell disease, paroxysmal nocturnal hemoglobinuria, and banked blood, and discuss therapeutics in development to target nitric oxide in the treatment of some of these disorders."
09/01/2010 - "The recent approvals of anti-C5 for the treatment of paroxysmal nocturnal hemoglobinuria as well as of C1 inh for the treatment of hereditary angioedema beyond European countries have provided a resurgence of interest in the potential of complement therapeutics for the treatment of disease."
12/01/2015 - "In view of the clinical experience acquired with drugs licensed for the treatment of rare diseases such as hereditary angioedema and paroxysmal nocturnal hemoglobinuria, growing evidence supports the safety and efficacy of complement therapeutics in restoring immune balance and preventing aggravation of clinical outcomes. "
03/01/2012 - "Thrombolytic therapy is effective in paroxysmal nocturnal hemoglobinuria: a series of nine patients and a review of the literature."
03/01/2012 - "We reviewed the outcome of thrombolytic therapy for patients with paroxysmal nocturnal hemoglobinuria who had thromboses refractory to anticoagulation at our institutions. "
05/01/2003 - "Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome."
10/01/1985 - "Thrombolytic therapy for inferior vena cava thrombosis in paroxysmal nocturnal hemoglobinuria."