Wiskott-Aldrich syndrome
ICD-10 D82.0 is a billable code used to indicate a diagnosis of wiskott-aldrich syndrome.
Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive immunodeficiency disorder characterized by the triad of eczema, recurrent infections, and thrombocytopenia (low platelet count). This condition arises due to mutations in the WAS gene, which plays a crucial role in the function of immune cells and platelets. Patients with WAS often experience a range of hematologic complications, including increased susceptibility to infections due to impaired antibody production and T-cell function. The thrombocytopenia can lead to bleeding disorders, making management of minor injuries challenging. Additionally, individuals with WAS are at an elevated risk for autoimmune diseases and malignancies, particularly lymphomas. Diagnosis typically involves clinical evaluation, family history, and genetic testing to confirm mutations in the WAS gene. Management strategies focus on supportive care, including immunoglobulin replacement therapy, platelet transfusions, and in severe cases, hematopoietic stem cell transplantation. Early diagnosis and intervention are critical to improving outcomes and quality of life for affected individuals.
Detailed history of symptoms, family history of WAS, and results of genetic testing.
Management of recurrent infections, eczema treatment, and monitoring of platelet levels.
Documentation must reflect the ongoing management of complications and response to therapies.
Comprehensive blood work results, including platelet counts and any transfusion records.
Evaluation of bleeding episodes and management of thrombocytopenia.
Must document any co-existing hematologic disorders and their management.
Used when a patient with WAS requires platelet transfusion due to severe thrombocytopenia.
Document the indication for transfusion and the patient's platelet count.
Hematology specialists should ensure accurate coding of the transfusion procedure.
The key symptoms include eczema, recurrent infections, and thrombocytopenia. These symptoms typically manifest in early childhood.
