Cooley's anemia

noun

Coo·​ley's anemia ˈkü-lēz- 

: an inherited disorder of hemoglobin synthesis that is the most severe form of beta-thalassemia, is marked by severe anemia associated with deficient hemoglobin, the presence of microcytes, and accelerated red cell destruction, and is associated with enlargement of the liver and spleen, an increase in erythroid bone marrow, bone deformities, and poor growth rate

Note: Cooley's anemia is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene located on the chromosome numbered 11. Cooley's anemia occurs especially in individuals of Mediterranean ancestry and necessitates regular blood transfusions throughout life.

Word History

Etymology

Thomas B. Cooley †1945 American pediatrician

First Known Use

1934, in the meaning defined above

Medical Definition

Cooley's anemia

noun

Coo·​ley's anemia ˌkü-lēz- 

variants also Cooley's disease

: an inherited disorder of hemoglobin synthesis that is the most severe form of beta-thalassemia, is marked by severe anemia associated with deficient hemoglobin, the presence of microcytes, and accelerated red cell destruction, and is associated with enlargement of the liver and spleen, an increase in the erythroid bone marrow, bone deformities, and poor growth rate

called also beta-thalassemia major, thalassemia major

Note: Cooley's anemia is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene located on the chromosome numbered 11. Cooley's anemia occurs especially in individuals of Mediterranean ancestry and necessitates regular blood transfusions throughout life.