Results 21 to 30 of about 13,066 (196)
Background A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example,
Anna M. Jankowska +7 more
doaj +1 more source
Paroxysmal nocturnal hemoglobinuria
The aim is to report on recent observations related to the natural history of paroxysmal nocturnal hemoglobinuria (PNH) and to review new therapeutic strategies for controlling the hemolysis of PNH.This review focuses on studies designed to characterize the long-term outcome of patients with PNH treated with eculizumab and to define the relationship ...
openaire +7 more sources
Paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome are diseases of excess activation of the alternative pathway of complement that are treated with eculizumab, a humanized monoclonal antibody against the terminal complement ...
Xuan Yuan +8 more
doaj +1 more source
Positive predictive value of diagnosis coding for hemolytic anemias in the Danish National Patient Register [PDF]
PURPOSE: The nationwide public health registers in Denmark provide a unique opportunity for evaluation of disease-associated morbidity if the positive predictive values (PPVs) of the primary diagnosis are known.
Frederiksen, Henrik +3 more
core +2 more sources
Paroxysmal nocturnal hemoglobinuria in systemic lupus erythematosus: a case report
Introduction Paroxysmal nocturnal hemoglobinuria is an acquired disorder of hemopoiesis and is characterized by recurrent episodes of intravascular hemolysis due to an increased sensitivity to complement-mediated hemolysis.
Nakamura Norio +11 more
doaj +1 more source
Aplastic Crisis as Primary Manifestation of Systemic Lupus Erythematosus [PDF]
Aplastic crisis is an unusual feature of systemic lupus erythematosus (SLE). We report the case of a 54-year-old woman presenting with both (extravascular) Coombs-positive hemolytic anemia and laboratory findings of bone marrow hyporegeneration with ...
Alger M +23 more
core +1 more source
How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core +1 more source
Background Clones of glycosylphosphatidylinositol-anchor protein-deficient cells are characteristic in paroxysmal nocturnal hemoglobinuria and are present in about 40–50% of patients with severe aplastic anemia.
Phillip Scheinberg +3 more
doaj +1 more source
Paroxysmal Nocturnal Hemoglobinuria [PDF]
Abstract 1. Paroxysmal noctural hemoglobinuria is believed to be an acquired disease of the hematopoietic system in which abnormal red cells, white cells, and platelets are produced. The lesion of the cells probably involves the stromal proteins in such a fashion that they are susceptible to the proteolytic effect of a sytem of normal ...
openaire +2 more sources
Objective: To discuss the implementation of technical advances in laboratory diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria for validation of high-sensitivity flow cytometry protocols. Methods: A retrospective study based on analysis of
Rodolfo Patussi Correia +11 more
doaj +1 more source

