1/3. Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constant Spring (Hb CS/CS): a case report.Acute haemolysis associated with mild upper respiratory tract infection was observed in a Thai boy who presented with a rapid decline in haemoglobin (Hb) levels, haemoglobinuria and evidence of intravascular haemolysis. Several possible causes giving rise to such a condition were excluded including G6PD deficiency, which is extremely common in thailand. Subsequent haematological and molecular analyses demonstrated that the patient was homozygous for Hb Constant Spring (Hb CS/CS), an a globin haemoglobinopathy. It has been shown previously that patients with homozygous Hb CS had mild haemolytic anaemia secondary to an accumulation of alpha(CS) chains, which are toxic to red blood cell membrane cytoskeletons. Increased body temperature might induce more precipitation of this a globin variant. This report highlights the importance of Hb CS/CS as a potential predisposing cause of acute haemolysis in children that might be aggravated by acute bacterial or viral infections. This is particularly relevant for patients of Southeast Asian descent where this abnormal haemoglobin is highly prevalent.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/3. methyldopa, intravascular haemolysis and renal disease. A case report.A 74-year-old man, who was receiving methyldopa to control systemic hypertension, presented to hospital in biventricular cardiac failure. He was found to be severely anaemic and jaundiced as a result of acute intravascular haemolysis with prominent haemoglobinuria. The cardiac failure and anaemia were initially refractory to fluid restriction and diuretics, but responded to red cell transfusion. Renal failure supervened, probably on the basis of hypoperfusion as a consequence of diuresis and hypotension. The patient died suddenly, possibly from myocardial infarction. Acute intravascular destruction of red cells in association with methyldopa appears not to have been previously reported.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
3/3. Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara.We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |