Sickle-cell thalassemia beta zero without crisis
ICD-10 D57.42 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero without crisis.
Sickle-cell thalassemia beta zero is a genetic blood disorder characterized by the presence of both sickle hemoglobin (HbS) and thalassemia, specifically beta-thalassemia. This condition results from mutations in the HBB gene, leading to reduced or absent production of beta-globin chains. Patients with this disorder typically exhibit symptoms of hemolytic anemia, including fatigue, pallor, and jaundice, due to the destruction of sickle-shaped red blood cells. Unlike sickle cell disease, patients with sickle-cell thalassemia beta zero may have a milder phenotype, especially when not experiencing a vaso-occlusive crisis. The absence of crisis indicates that the patient is not currently experiencing acute pain episodes or complications related to sickle cell disease, which can include acute chest syndrome or stroke. Management often involves regular monitoring, blood transfusions, and hydroxyurea therapy to reduce the frequency of complications. Genetic counseling is also recommended for affected individuals and their families to understand inheritance patterns and reproductive options.
Detailed lab results showing hemoglobin levels and types, patient history of crises, and treatment plans.
Management of chronic anemia, monitoring for complications, and genetic counseling.
Ensure clear documentation of the absence of crisis and any related symptoms.
Family history of sickle cell disease or thalassemia, genetic test results, and counseling notes.
Genetic testing for family members, counseling for reproductive options.
Documentation must reflect the genetic basis of the condition and implications for family members.
Used to monitor hemoglobin levels and assess anemia severity.
Document the reason for the CBC and any relevant symptoms.
Hematology specialists should ensure that all lab results are clearly linked to the diagnosis.
Sickle-cell thalassemia beta zero is a specific form of sickle cell disease characterized by the presence of both sickle hemoglobin and beta-thalassemia. It typically presents with milder symptoms and fewer complications compared to other forms of sickle cell disease, especially when not in crisis.
