Sickle-cell thalassemia beta zero with splenic sequestration
ICD-10 D57.432 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with splenic sequestration.
Sickle-cell thalassemia beta zero with splenic sequestration is a complex hemolytic anemia resulting from the combination of sickle cell disease and beta-thalassemia. This condition arises due to mutations in the HBB gene, leading to the production of abnormal hemoglobin (HbS) and reduced beta-globin chains. Patients with this condition experience episodes of splenic sequestration, where sickled red blood cells become trapped in the spleen, causing acute splenic enlargement and potentially severe anemia. Symptoms may include pain crises, fatigue, jaundice, and splenomegaly. The management of this condition often requires a multidisciplinary approach, including regular blood transfusions, hydroxyurea therapy, and monitoring for complications such as infections and acute chest syndrome. Genetic counseling is also essential for affected families, as this condition is inherited in an autosomal recessive pattern. Understanding the interplay between sickle cell disease and thalassemia is crucial for effective treatment and management.
Detailed history of hemolytic episodes, laboratory results, and treatment plans.
Management of pain crises, blood transfusions, and monitoring for splenic sequestration.
Ensure accurate documentation of genetic testing and family history.
Genetic testing results, family pedigree, and counseling notes.
Counseling families about inheritance patterns and risks for future offspring.
Document the implications of genetic findings on treatment and management.
Used during acute splenic sequestration episodes requiring transfusion.
Document the reason for transfusion and patient response.
Hematology specialists should ensure accurate coding of transfusion episodes.
Splenic sequestration is a critical complication that can lead to acute anemia and requires immediate medical attention. It is essential to document these episodes accurately for proper management and coding.
