ICD-10 Specific code D57.2: Double heterozygous sickling disorders

Specific codes in ICD-10 are unique alphanumeric designations used to identify and categorize diseases, disorders, and conditions. They consist of 3-5 characters, including both letters and numbers, that provide a high level of detail and specificity.

Translations

Language Translation
Flag for English language  English Double heterozygous sickling disorders
Flag for French language  French Affections à hématies falciformes [drépanocytaires] hétérozygotes doubles

Hierarchical position

Level Code Title
1 III Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
2 D55-D59 Haemolytic anaemias
3 D57 Sickle-cell disorders
4 D57.2 Double heterozygous sickling disorders

Indicated medicines

Active Ingredient Description
Exagamglogene autotemcel

Exagamglogene autotemcel is a cell therapy consisting of autologous CD34+ HSPCs ex vivo edited by CRISPR/Cas9-technology. The highly specific guide RNA enables CRISPR/Cas9 to make a precise DNA double-strand break at the critical transcription factor binding site (GATA1) in the erythroid specific enhancer region of the BCL11A gene. As a result of the editing, GATA1 binding is irreversibly disrupted and BCL11A expression reduced. Reduced BCL11A expression results in an increase in γ-globin expression and foetal haemoglobin (HbF) protein production in erythroid cells, addressing the absent globin in transfusion-dependent β-thalassemia (TDT) and the aberrant globin in sickle cell disease (SCD), which are the underlying causes of disease.

Voxelotor

Voxelotor is a haemoglobin S (HbS) polymerisation inhibitor that binds to HbS with a 1:1 stoichiometry and exhibits preferential partitioning to red blood cells (RBCs). By increasing the affinity of Hb for oxygen, voxelotor demonstrates dose-dependent inhibition of HbS polymerisation. Voxelotor inhibits RBC sickling and improves RBC deformability.