| 释义 |
drepanocytic anemia
Thesaurus| Noun | 1.drepanocytic anemia - a congenital form of anemia occurring mostly in blacks; characterized by abnormal blood cells having a crescent shapecrescent-cell anaemia, crescent-cell anemia, drepanocytic anaemia, sickle-cell anaemia, sickle-cell anemia, sickle-cell diseasemonogenic disease, monogenic disorder - an inherited disease controlled by a single pair of genesanemia, anaemia - a deficiency of red blood cells |
EncyclopediaSeeSickle Cell Anemiadrepanocytic anemia
sick·le cell a·ne·mi·a [MIM*603903] an autosomal recessive anemia characterized by crescent- or sickle-shaped erythrocytes and accelerated hemolysis, due to substitution of a single amino acid (valine for glutamic acid) in the sixth position of the β-chain of hemoglobin, the gene of which is on chromosome 11; affected homozygotes have 85-95% hemoglobin (Hb) S and severe anemia, whereas heterozygotes (said to have sickle cell trait) have 40-45% Hb S, the rest being normal Hb A; low oxygen tension causes polymerization of the abnormal β-chains, thus distorting the shape of the red blood cells to the sickle form. Homozygotes develop "crisis" episodes of severe pain due to microvascular occlusions, bone infarcts, leg ulcers, and atrophy of the spleen associated with increased susceptibility to bacterial infections, especially streptococcal pneumonia. Occurs most commonly in people of African descent. Synonym(s): drepanocytic anemia, sickle cell diseasesick·le cell a·ne·mi·a (sik'ĕl sel ă-nē'mē-ă) An autosomal dominant anemia characterized by crescentic or sickle-shaped erythrocytes and by accelerated hemolysis, due to substitution of a single amino acid (valine for glutamic acid) in the sixth position of the beta chain of hemoglobin (Hb); affected homozygotes have 85-95% Hb S and severe anemia, whereas heterozygotes (said to have sickle cell trait) have 40-45% Hb S, the rest being normal Hb A; low oxygen tension causes polymerization of the abnormal beta chains, thus distorting the shape of the red blood cells to the sickle form. Homozygotes develop "crises": episodes of severe pain due to microvascular occlusions, bone infarcts, leg ulcers, and atrophy of the spleen associated with increased susceptibility to bacterial infections, especially streptococcal pneumonia. Occurs almost exclusively in blacks.
Synonym(s): crescent cell anemia, drepanocytic anemia, sickle cell disease. drepanocytic anemia
Related to drepanocytic anemia: drepanocytic anaemiaSynonyms for drepanocytic anemianoun a congenital form of anemia occurring mostly in blacksSynonyms- crescent-cell anaemia
- crescent-cell anemia
- drepanocytic anaemia
- sickle-cell anaemia
- sickle-cell anemia
- sickle-cell disease
Related Words- monogenic disease
- monogenic disorder
- anemia
- anaemia
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