has clinical code has causal or contributing genetic variation D57 Sickle-Cell Disorders Hemoglobinopathy Other HBB Variants HBB, GLU6VAL Sickle Cell Disease Sickle Cell Disorder Sickle cell disease (SCD) is a pleiotropic inherited disorder of the blood, characterised by the appearance of sickle-shaped red blood cells and anemia. It results from homozygosity for the sickle beta-globin gene mutation at position 6 (glu > val), or compound heterozygosity for the sickle gene and either another mutation for a different hemoglobin variant or one of numerous beta-thalassemia mutations. Drepanocytosis Sickle Hemoglobinopathy SCD Banana Cell Disease Sickle-Cell Disorder Sickle Cell Hemoglobinopathy Few but definitions not specific enough Suggest update to description in NCIT. Sickle cell diseases include inheritance of the gene for sickle hemoglobin from one parent and one of the following: A gene for sickle hemoglobin from the other parent (Hb SS), A gene for another abnormal hemoglobin from the other parent (Hb SC, HbSD Punjab, HbSO Arab, HbS Leopore, or Hb SE), A gene that limits hemoglobin production from the other parent (sickle beta +/0 thalassemia). In S beta (+) thalassemia there is some production of beta globin, and in S beta (0) thalassemia there is no production of beta globin.