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pure red cell anaemia Congenital nonregenerative, familial hypoplastic, or pure red cell anaemia; erythrogenesis imperfecta; Diamond-Blackfan syndrome; autosomal recessive normocytic normochromic anaemia resulting from congenital hypoplasia of the bone marrow, which is grossly deficient in erythroid precursors while other elements are normal; anaemia is progressive and severe, but leukocyte and platelet counts are normal or slightly reduced; survival of transfused erythrocytes is normal; minor congenital anomalies are found in some patients.
Synonym: congenital nonregenerative anaemia, Diamond-Blackfan anaemia, Diamond-Blackfan syndrome, erythrogenesis imperfecta, familial hypoplastic anaemia, pure red cell anaemia.
(05 Mar 2000)
sickle cell anaemia <haematology> Disease common in races of people from areas in which malaria is endemic.
The cause is a point mutation in the allele that codes for the beta chain of haemoglobin with a substitution of (valine for glutamic acid at position 6. The defective haemoglobin (HbS) crystallizes readily at low oxygen tension.
In consequence, erythrocytes from homozygotes change from the normal discoid shape to a sickled shape when the oxygen tension is low and these sickled cells become trapped in capillaries or damaged in transit, leading to severe anaemia.
In heterozygotes, the disadvantages of the abnormal haemoglobin are apparently outweighed by increased resistance to Plasmodium falciparum malaria, probably because parasitised cells tend to sickle and are then removed from circulation.
Symptoms include joint pain, acute abdominal pain, and ulcerations of the lower extremities.
Origin: Gr. Haima = blood
(18 Nov 1997)
sickle cell anaemia: bone manifestations <radiology> 8-13% of blacks carry sickling factor, symptoms: chronic ulcers, pain crises, many infections, priapism X-ray findings: deossification due to marrow hyperplasia, decreased bone density in skull with widened diploe, H-shaped vertebrae or fish vertebrae, rib notching, thrombosis and infarction, avascular necrosis, especially femoral head, periosteal treatmentn (bone within bone), secondary osteomyelitis, Staph. Aureus greater than Salmonella, dactylitis = hand foot syndrome, growth effects, bone shortening secondary to diminished blood supply, death less than 40y
(12 Dec 1998)
sideroblastic anaemia <haematology> A form of refractory anaemia caused by sideroblasts in the bone marrow.
Origin: Gr. Haima = blood
(09 Oct 1997)
hyperchromic anaemia <haematology> A type of anaemia which results when the body lacks sufficient vitamin B and the resulting red blood cells are thicker than normal, appearing more deeply coloured.
Origin: Gr. Haima = blood
(11 Jan 1998)
hypochromic anaemia 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.
(05 Mar 2000)
hypochromic microcytic anaemia Anaemia due to iron deficiency or thalassaemia, and characterised by lower than normal mean corpuscular volume, mean corpuscular haemoglobin, and mean corpuscular haemoglobin concentration.
(05 Mar 2000)
hypoferric anaemia Hypochromic microcytic anaemia characterised by low serum iron, increased serum iron-binding capacity, decreased serum ferritin, and decreased marrow iron stores.
Synonym: hypoferric anaemia.
(05 Mar 2000)
hypoplastic anaemia <haematology> A low red blood cell count that results from the underproduction of red blood cells by the bone marrow. This is often secondary to a drug (chemotherapy) side effect.
(27 Sep 1997)
hypoproliferative anaemia <haematology> Anaemia which results from abnormally low levels of red blood cells and haemoglobin in the blood. It is caused because the person has too few of the stem cells which make the red blood cells.
(09 Oct 1997)
slaty anaemia An ash-gray pallor in poisoning from acetanelid or silver (argyria).
(05 Mar 2000)
spastic anaemia Local anaemia resulting from nontransitory contraction of the arterial vessels in the affected region.
(05 Mar 2000)
spherocytic anaemia <haematology> A hereditary disorder that leads to a chronic haemolytic anaemia due to an abnormality in the red blood cell membrane.
This disorder is caused by a defective gene. Red cells are resistant to stress and rupture easily. Infants may appear jaundiced and pale. Fatigue, weakness and shortness of breath are other symptoms that may be seen in older patients. The spleen may also be enlarged.
Treatment includes splenectomy (removal of the spleen). After this is accomplished the life-span of the red blood cells returns to normal.
(27 Sep 1997)
splenic anaemia <syndrome> Chronic congestive splenomegaly that occurs primarily in children as a sequel to hypertension in the portal or splenic veins, usually as a result of thrombosis of the veins; anaemia, splenomegaly, and irregular episodes of gastrointestinal bleeding are usually observed, with ascites, jaundice, leukopenia, and thrombocytopenia developing in various conbinations.
Synonym: Banti's disease, splenic anaemia.
(05 Mar 2000)
neonatal anaemia <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)
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