Sickle-cell/Hb-C disease with splenic sequestration
ICD-10 D57.212 is a billable code used to indicate a diagnosis of sickle-cell/hb-c disease with splenic sequestration.
Sickle-cell/Hb-C disease with splenic sequestration is a complex hemolytic anemia characterized by the presence of both sickle hemoglobin (HbS) and hemoglobin C (HbC). This condition arises from mutations in the HBB gene, leading to the production of abnormal hemoglobin that distorts red blood cells into a sickle shape, causing them to become rigid and sticky. In splenic sequestration, these sickled cells can obstruct blood flow in the spleen, leading to acute splenic enlargement and a sudden drop in hemoglobin levels. Patients may present with symptoms such as severe abdominal pain, fatigue, pallor, and signs of shock due to rapid blood loss. The condition is particularly prevalent in individuals of African and Mediterranean descent. Management often involves hydration, pain control, and blood transfusions, with the potential for splenectomy in recurrent cases. Understanding the genetic basis and clinical implications of this disease is crucial for effective treatment and management.
Detailed lab results, genetic testing outcomes, and clinical notes on splenic function.
Management of acute splenic sequestration crises and chronic anemia management.
Ensure comprehensive documentation of all symptoms and treatments to support coding.
Growth and development assessments, family history of hemoglobinopathies.
Routine monitoring of children with sickle-cell disease and management of pain crises.
Documenting developmental milestones and any complications related to sickle-cell disease.
Used during acute splenic sequestration management.
Document the need for transfusion due to anemia severity.
Hematology specialists should ensure transfusion protocols are followed.
Splenic sequestration is a critical complication of sickle-cell disease that can lead to severe anemia and requires immediate medical attention. It is characterized by the trapping of sickled red blood cells in the spleen, leading to splenomegaly and a rapid drop in hemoglobin levels.
