The role of the C' 1 system in the hemolysis of PNHE has been emphasized recently by the demonstration in this laboratory that PNHE lysis and classical C'-dependent immune lysis have similar cation requirements and pH optima (2, 3). In addition, the enhancing effects of C'la, C'1 esterase, and C'l activators upon PNHE hemolysis suggested that the early steps of C'activation ultimately leading t...

By J. V. DAdE, M.B., M.R.C.P. LONDON H YDROGEN ion concentration has a controlling effect upon many hemolytic systems, both simple and complex. Osbor& in 1934 reviewed the early literature on the effect of pH on the hemolysis by complement of corpuscles sensitized by hemolytic immune body. He found that the optimum reaction for the hemolysis of sheep corpuscles by guinea pig serum was about pH ...

Ten consecutive patients with myeloysis. One patient had a positive sucrose fibrosis were examined for the following hemolysis test and low red cell ACHE. signs of PNH: Ham’s test, the sucrose The remaining patients had no signs of hemolysis test, low red cell acetylcholinPNH. In two of the patients, the PNH esterase (ACHE) activity and intrasigns disappeared after splenectomy. The vascular hem...

The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the alternative pathway of complement. This may explain some of the complexi...

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thromb...

BACKGROUND Aplastic anemia may develop during pregnancy and sometimes improves spontaneously after delivery. The effects of pregnancy on aplastic anemia after immunosuppressive treatment and of aplastic anemia on the outcome of pregnancy have not been described. OBJECTIVE To determine the outcome of pregnancy and the disease course among women with aplastic anemia who received immunosuppressi...

Aberrant smooth muscle dystonia during hemolytic episodes in paroxysmal nocturnal hemoglobinuria (PNH) is implicated in the symptoms of abdominal pain, dysphagia and erectile dysfunction. Here we report two PNH patients treated with the complement inhibitor, eculizumab. Complement inhibition has been sustained for over 2 years and results in resolution of intravascular hemolysis and amelioratio...

Blackwater fever is characterized by acute intravascular hemolysis with hemoglobinuria in patients with Plasmodium falciparum malaria. Its pathogenesis and management are still debated. Nine cases of this syndrome occurred in 2003 at Kiremba Hospital in Burundi in children receiving multiple quinine treatments.

Flood diuresis so far lowers the renal threshold for hemoglobin that the pigment appears in quantity in the urine as result of a hemoglobinemia insufficient under ordinary circumstances to lead to the elimination of even a trace of it. In pathological conditions that involve blood destruction hemoglobin probably passes into the tubules much more often than it reaches the urine, being prevented ...