Sickle-cell thalassemia beta plus with splenic sequestration
ICD-10 D57.452 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta plus with splenic sequestration.
Sickle-cell thalassemia beta plus with splenic sequestration is a complex hematological condition characterized by the presence of both sickle cell disease and beta-thalassemia. This condition arises from mutations in the HBB gene, leading to abnormal hemoglobin production. Patients with this disorder experience episodes of hemolytic anemia due to the sickling of red blood cells, which can obstruct blood flow and cause pain crises. The 'beta plus' designation indicates that some normal beta-globin is produced, but not enough to prevent anemia. Splenic sequestration occurs when sickled cells become trapped in the spleen, leading to splenomegaly and acute drops in hemoglobin levels. This can result in severe complications, including hypovolemic shock. Management often requires blood transfusions, hydroxyurea therapy, and close monitoring for splenic function. Genetic counseling is also essential for affected families, as this condition is inherited in an autosomal recessive pattern.
Detailed patient history, including family history of hemoglobinopathies, laboratory results showing hemoglobin levels, and evidence of splenic sequestration.
Management of pain crises, blood transfusion therapy, and monitoring for splenic complications.
Ensure accurate coding of both sickle cell disease and thalassemia, and document any acute complications.
Genetic testing results, family pedigree charts, and counseling notes.
Counseling families about inheritance patterns and risks of having affected children.
Document the specific mutations identified and their implications for treatment.
Used when a patient with D57.452 requires a blood transfusion due to severe anemia.
Document the indication for transfusion, including hemoglobin levels and symptoms.
Hematology specialists should ensure that transfusion protocols are followed.
Splenic sequestration is a critical complication that can lead to severe anemia and requires immediate medical attention. It occurs when sickled red blood cells become trapped in the spleen, causing splenomegaly and a rapid drop in hemoglobin levels.
