1/23. Myeloneuropathy following nitrous oxide anesthaesia in a patient with macrocytic anaemia.The neurological condition triggered by anaesthesia with nitrous oxide involves the cyanocobalamine pathway and is characterised by progressive demyelination and axonal lesions of the peripheral nerves and cervicothoracic spinal cord (posterior and anterolateral columns) giving a peripheral neuropathy and very frequently subacute combined degeneration of the spinal cord. It is possible to show these demyelinating lesions by MRI of the spine, allowing early diagnosis and follow-up. We describe a case of myeloneuropathy with onset a few hours after nitrous oxide anaesthesia in a patient with macrocytic anaemia and possible subclinical vitamin B(12) deficiency and MRI evidence of a lesion of the cervical spinal cord. Neurological and haematological improvement followed cyanocobalamine replacement.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/23. hypothyroidism associated with myeloid neoplasia. About 2 cases.We report 2 cases of hypothyroidism in whom the associated haematological disturbances (macrocytic anaemia in both cases, mild leukopenia and thrombopenia in one patient) failed to be corrected by thyroid hormone replacement therapy. Further investigations led to the diagnosis of acute myeloid leukaemia (AML) in the first patient and myelodysplastic syndrome (MDS) in the other. The reasons of the delayed diagnosis and the possible mechanisms explaining this rare combination are discussed. The hypothesis of a purely coincidental association seems most likely.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
3/23. Severe CF manifestation with anaemia and failure to thrive in a 394delTT homozygous patient.We report on a 394delTT homozygous cystic fibrosis (CF) patient with severe disease progression. At the diagnosis made at the age of 2.5 months, he suffered from macrocytic anaemia as the most prominent symptom of CF, malnutrition, hypoproteinaemia and profound hypoalbuminaemia, but demonstrated only minimal pulmonary symptoms. Abnormal sweat chlorides confirmed the diagnosis of CF. Severe pulmonary and liver disease caused death after 6 years.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/23. Remission of transfusion-dependent myelodysplastic syndrome in association with respiratory tract infection.We describe a case of blood transfusion-dependent myelodysplastic syndrome (refractory anaemia), associated with macrocytosis and elevated percentage of hypochromic cells. Following an acute hospital admission with a respiratory tract infection, the patient entered a complete and sustained remission.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
5/23. Acquired copper deficiency following prolonged jejunostomy feeds.A 19-year-old man who developed extensive oesophageal lye (Alkali) stricture and received long-term enteral nutrition (eight months) with a jejunostomy tube developed macrocytic anaemia (Hb: 41 g/L) with leucopenia (white blood cell [WBC]: 3.0 x 10(9)/L). The patient's serum vitamin B12, folate, iron and liver function tests were normal. bone marrow examination revealed gross erythroid hyperplasia and cytoplasmic vacuolization of erythroid and myeloid elements. Further investigations revealed low serum copper (0.3 micromol/L) and ceruloplasmin concentrations (<30 mg/L) with marginally low normal serum concentration of red cell peroxidase (13 U/gHb), establishing the diagnosis of copper deficiency anaemia. The anaemia and leucopenia responded intermittently to intravenous copper therapy, but the serum copper concentration dropped when intravenous copper therapy was withdrawn. Enteral jejunostomy copper supplementation failed to maintain adequate serum copper concentrations. After stabilizing the general condition of the patient, a pharyngo-gastric anastamosis was performed and normal oral diet commenced, which restored normal serum copper concentration. This case report suggests that copper supplements in the form of copper sulphate are not adequately absorbed when administered through a jejunostomy tube.- - - - - - - - - - ranking = 0.6keywords = anaemia (Clic here for more details about this article) |
6/23. Imerslund-Grasbeck syndrome in a Libyan boy.Immerslund-Grasbeck syndrome is an uncommon disease, characterized by megaloblastic anaemia and persistent proteinuria. A Libyan boy with the characteristic findings is presented. He received intramuscular vitamin B12 injections and there followed a remarkable clinical and haematological improvement.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
7/23. Studies on thiamine metabolism in thiamine-responsive megaloblastic anaemia.We have investigated thiamine metabolism and transport in the erythrocytes of two patients from unrelated families with thiamine responsive megaloblastic anaemia associated with diabetes mellitus and sensorineural deafness. Both patients had low concentrations of thiamine compounds in plasma and red blood cells. When erythrocytes were incubated with thiazole-[2-14C]-thiamine or [35S]-thiamine in vitro, the concentration of label within the cells was markedly reduced compared with controls. In addition, thiamine pyrophosphokinase activity was deficient in haemolysates prepared from the patients. Some relatives of the patients showed abnormal parameters of thiamine status and transport. In both patients treatment with a lipophilic compound corrected the haematological abnormalities and diabetes and in one patient has so far prevented the progression of deafness. We propose that the disorder is caused by an inherited defect of thiamine transport, possibly related to deficient pyrophosphokinase activity, leading to intracellular depletion of active thiamine metabolite derivatives.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
8/23. Megaloblastic anaemia due to sulphasalazine responding to drug withdrawal alone.A 60 year old man with ulcerative colitis since 1975 and treated with sulphasalazine, presented with severe megaloblastic anaemia caused by folate deficiency. The drug was stopped, and the anaemia recovered promptly. There was no relapse one year later.- - - - - - - - - - ranking = 1.2keywords = anaemia (Clic here for more details about this article) |
9/23. Vitamin B12 and brain development.This is a report of a 1 1/2-year-old male with megaloblastic anaemia and a progressive neurological disorder clinically resembling leucodystrophy. The child was exclusively breast fed and his mother had been a strict vegetarian for more than 4 years before the child was born. After Vitamin B12 therapy the anaemia disappeared and the neurological condition dramatically improved.- - - - - - - - - - ranking = 0.4keywords = anaemia (Clic here for more details about this article) |
10/23. Macrocytic anaemia due to copper deficiency in a patient with late onset hypogammaglobulinaemia.Very few cases of acquired severe copper deficiency have been described. The principal effects are haematological, but the precise abnormalities are uncertain due to the possible association of other deficiencies. A case of isolated severe copper deficiency associated with late onset hypogammaglobulinaemia is reported in which the chief findings were macrocytic anaemia, neutropenia and a decrease in mean platelet volume. All these abnormalities resolved when copper therapy was instituted and recurred when the medication was stopped.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
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