Lymphocyte function antigen-1 [LFA-1] defect
ICD-10 D84.0 is a billable code used to indicate a diagnosis of lymphocyte function antigen-1 [lfa-1] defect.
Lymphocyte function antigen-1 (LFA-1) defect is a rare immunodeficiency disorder characterized by the inability of leukocytes to adhere properly to endothelial cells and migrate to sites of infection or inflammation. This defect is primarily due to mutations in the ITGAL gene, which encodes the alpha subunit of the LFA-1 integrin. Patients with LFA-1 defect often present with recurrent bacterial infections, particularly with encapsulated organisms, due to impaired immune response. The condition can lead to severe complications, including chronic lung disease, skin infections, and sepsis. Diagnosis is typically confirmed through flow cytometry, which reveals decreased expression of LFA-1 on leukocytes. Management includes prophylactic antibiotics, immunoglobulin therapy, and in some cases, hematopoietic stem cell transplantation. Understanding the clinical implications and the hematologic context of this defect is crucial for effective coding and management of affected patients.
Detailed immunologic testing results, patient history of infections, and treatment plans.
Patients presenting with recurrent infections, particularly in childhood, and those requiring immunoglobulin therapy.
Ensure that all laboratory results are clearly documented and correlate with clinical findings.
Complete blood counts, flow cytometry results, and any relevant bone marrow biopsy findings.
Patients with hematologic manifestations of immunodeficiency, such as leukopenia or lymphopenia.
Documentation should include any hematologic abnormalities that may complicate the patient's condition.
Used to evaluate the patient's hematologic status in conjunction with LFA-1 defect.
Document the reason for the CBC and any relevant findings.
Immunologists should correlate CBC results with immunologic evaluations.
Common symptoms include recurrent bacterial infections, particularly with encapsulated organisms, chronic lung disease, and skin infections.
Diagnosis is typically made through flow cytometry, which shows decreased expression of LFA-1 on leukocytes, along with genetic testing for mutations in the ITGAL gene.
