| anaemia, Cooley's | Better known today as thalassaemia (or as beta thalassaemia or thalassaemia major).The clinical picture of this important type of anaemia was first described in 1925 by the paediatrician Thomas Benton Cooley. Another name for the disease is Mediterranean anaemia. The name thalassaemia was coined by the Nobel Prise winning pathologist George Whipple and the professor of paediatrics Wm Bradford at Univ. Of Rochester because thalassa in Greek means the sea (like the Mediterrranean Sea) + -aemia means in the blood so thalassaemia means sea in the blood. Thalassaemia is not just one disease. It is a complex contingent of genetic (inherited) disorders all of which involve underproduction of haemoglobin, the indispensable molecule in red blood cells that carries oxygen. The globin part of normal adult haemoglobin is made up of 2 alpha and 2 beta polypeptide chains. In beta thalassaemia, there is a mutation (change) in both beta globin chains leading to underproduction (or absence) of beta chains, underproduction of haemoglobin, and profound anaemia. The gene for beta thalassaemia is relatively frequent in people of Mediterranean origin (for example, from Italy and Greece). Children with this disease inherit one gene for it from each parent. The parents are carriers (heterozygotes) with just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal. Their children affected with beta thalassaemia seem entirely normal at birth because at birth we still have predominantly foetal haemoglobin which does not contain beta chains. The anaemia surfaces in the first few months after birth and becomes progressively more severe leading to pallor and easy fatiguability, failure to thrive (grow), bouts of fever (due to infections) and diarrhoea. Treatment based on blood transfusions is helpful but not curative. Gene therapy will, it is hoped, be applicable to this disease. (12 Dec 1998) |
|---|---|
| anaemia, dyserythropoietic, congenital | A familial disorder characterised by anaemia with multinuclear erythroblasts, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors. Type II is the most common of the 3 types of congenital dyserythropoietic anaemia; it is often referred to as hempas, based on the hereditary erythroblast multinuclearity with positive acidified serum test. (12 Dec 1998) |
| anaemia gravis | <haematology> This form of anaemia occurs when the bone marrow ceases sufficient red and white blood cell production. It may be induced by exposures to high levels of toxic chemicals, radiation and certain drugs. It is generally unresponsive to specific therapy, often accompanied by granulocytopenia and thrombocytopenia, in which the bone marrow may not necessarily be hypocellular or hypoplastic but fails to produce adequate numbers of peripheral blood elements. The term actually is all inclusive and most probably encompasses several clinical syndromes. Origin: Gr. Haima = blood (29 Sep 1997) |
| anaemia, hypochromic | Anaemia characterised by a decrease in the ratio of the weight of haemoglobin to the volume of the erythrocyte, i.e., the mean corpuscular haemoglobin concentration is less than normal. The individual cells contain less haemoglobin than they could have under optimal conditions. Hypochromic anaemia may be caused by iron deficiency from a low iron intake, diminished iron absorption, or excessive iron loss. It can also be caused by infections or other diseases, therapeutic drugs, lead poisoning, and other conditions. (12 Dec 1998) |
| anaemia, iron deficiency | Deficiency of iron results in anaemia because iron is necessary to make haemoglobin, the key molecule in red blood cells responsible for the transport of oxygen. In iron deficiency anaemia, the red cells are unusally small (microcytic) and pale (hypochromic). Characteristic features of iron deficiency anaemia in children include failure to thrive (grow) and increased infections. The treatment of iron deficiency anaemia, whether it be in children or adults, is with iron and iron-containing foods. Food sources of iron include meat, poultry, eggs, vegetables and cereals (especially those fortified with iron). According to the National Academy of Sciences, the Recommended Dietary Allowances of iron are 15 milligrams per day for women and 10 milligrams per day for men. Anaemia characterised by low or absent iron stores, low serum iron concentration, elevated free erythrocyte porphorin, low transferrin saturation, elevated transferrin, low serum ferritin, low haemoglobin concentration or haematocrit, and hypochromic microcytic red blood cells. Symptoms may include pallor, angular stomatitis and other oral lesions, gastrointestinal complaints, retinal haemorrhages and exudates, and thinning and brittleness of the nails. Among the causes of iron-deficiency anaemia are inadequate iron intake, impaired iron absorption, increased blood loss and increased requirements such as infancy, pregnancy, and lactation. (12 Dec 1998) |
| anaemia, macrocytic | Anaemia characterised by larger than normal erythrocytes, increased mean corpuscular volume (mcv) and increased mean corpuscular haemoglobin (mch). (12 Dec 1998) |
| anaemia, mediterranean | Better known today as thalassaemia (or as beta thalassaemia or thalassaemia major).The clinical picture of this important type of anaemia was first described in 1925 by the paediatrician Thomas Benton Cooley. The name thalassaemia was coined by the Nobel Prise winning pathologist George Whipple and the professor of paediatrics Wm Bradford at U. Of Rochester because thalassa in Greek means the sea (like the Mediterrranean Sea) + -aemia means in the blood so thalassaemia means sea in the blood. Thalassaemia is not just one disease. It is a complex contingent of genetic (inherited) disorders all of which involve underproduction of haemoglobin, the indispensable molecule in red blood cells that carries oxygen. The globin part of normal adult haemoglobin is made up of 2 alpha and 2 beta polypeptide chains. In beta thalassaemia, there is a mutation (change) in both beta globin chains leading to underproduction (or absence) of beta chains, underproduction of haemoglobin, and profound anaemia. The gene for beta thalassaemia is relatively frequent in people of Mediterranean origin (for example, from Italy and Greece). Children with this disease inherit one gene for it from each parent. The parents are carriers (heterozygotes) with just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal. Their children affected with beta thalassaemia seem entirely normal at birth because at birth we still have predominantly foetal haemoglobin which does not contain beta chains. The anaemia surfaces in the first few months after birth and becomes progressively more severe leading to pallor and easy fatiguability, failure to thrive (grow), bouts of fever (due to infections) and diarrhoea. Treatment based on blood transfusions is helpful but not curative. Gene therapy will, it is hoped, be applicable to this disease. (12 Dec 1998) |
| anaemia, megaloblastic | Anaemia characterised by the presence of megaloblasts in the bone marrow. (12 Dec 1998) |
| anaemia, myelophthisic | Anaemia characterised by appearance of immature myeloid and nucleated erythrocytes in the peripheral blood, resulting from infiltration of the bone marrow by foreign or abnormal tissue. (12 Dec 1998) |
| anaemia, neonatal | The mildest form of erythroblastosis foetalis in which anaemia is the chief manifestation. (12 Dec 1998) |
| anaemia neonatorum | <haematology> A condition which develops in the foetus due to an incompatibility between the mother's blood type (RH factor) and the baby's. Maternal antibodies, which enter the foetal circulation during delivery attack the baby's red blood cells leading to haemolysis (rupture of the cells). Symptoms include an infant with an enlarged liver and spleen, swelling, jaundice and anaemia. (27 Sep 1997) |
| anaemia of chronic disease | <disease> A form of anaemia which develops as the result of a long-term infection or illness. Chronic diseases can interfere with red blood cell production in addition to shortening red blood cell life span in the body. Symptoms are largely due to the underlying disease. Haemoglobin and haematocrit are generally low. Iron studies may be low to normal. Red blood cell indices may usually normal. (27 Sep 1997) |
| anaemia, pernicious | A megaloblastic anaemia occurring in children but more commonly in later life, characterised by histamine-fast achlorhydria, in which the laboratory and clinical manifestations are based on malabsorption of vitamin b 12 due to a failure of the gastric mucosa to secrete adequate and potent intrinsic factor. (12 Dec 1998) |
| anaemia, refractory | Anaemia (a shortage of red blood cells) unresponsive to treatment. (12 Dec 1998) |
| anaemia, refractory, with excess of blasts | Chronic refractory anaemia with granulocytopenia, and/or thrombocytopenia. Myeloblasts and progranulocytes constitute 5 to 40 percent of the nucleated marrow cells. (12 Dec 1998) |