Sickle-cell thalassemia beta plus without crisis
ICD-10 D57.44 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta plus without crisis.
Sickle-cell thalassemia beta plus is a genetic blood disorder characterized by the presence of both sickle cell disease and beta-thalassemia traits. Patients with this condition have a combination of abnormal hemoglobin (hemoglobin S) and reduced production of beta-globin chains, leading to a mixed phenotype of sickle cell disease and thalassemia. Unlike sickle cell disease, which can present with painful crises due to vaso-occlusive events, patients with sickle-cell thalassemia beta plus may not experience these crises frequently or at all. The condition can lead to chronic hemolytic anemia, where red blood cells are destroyed faster than they can be produced, resulting in symptoms such as fatigue, pallor, and jaundice. Genetic factors play a crucial role, as this condition is inherited in an autosomal recessive pattern. Diagnosis typically involves hemoglobin electrophoresis to identify the types of hemoglobin present and genetic testing to confirm the presence of mutations in the HBB gene. Management focuses on monitoring hemoglobin levels, managing anemia, and preventing complications, with transfusions or hydroxyurea therapy considered in more severe cases.
Detailed lab results, genetic testing, and clinical notes on anemia symptoms.
Management of chronic anemia, monitoring for complications, and patient education.
Ensure clear documentation of the absence of crisis to avoid misclassification.
Genetic test results and family history of hemoglobinopathies.
Counseling patients on inheritance patterns and implications for family members.
Accurate recording of genetic mutations is crucial for proper coding.
Used to monitor hemoglobin levels in patients with sickle-cell thalassemia beta plus.
Document the reason for the CBC and any relevant symptoms.
Hematologists should ensure that CBC results are linked to the diagnosis.
Sickle-cell thalassemia beta plus involves a combination of sickle cell disease and beta-thalassemia traits, leading to chronic hemolytic anemia without frequent crises, while sickle-cell disease typically presents with recurrent painful crises.
Diagnosis is made through hemoglobin electrophoresis and genetic testing to identify the presence of hemoglobin S and mutations in the HBB gene.
