Autoimmune lymphoproliferative syndrome [ALPS]
ICD-10 D89.82 is a billable code used to indicate a diagnosis of autoimmune lymphoproliferative syndrome [alps].
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder characterized by the abnormal proliferation of lymphocytes, leading to lymphadenopathy, splenomegaly, and autoimmune manifestations. It is primarily caused by mutations in genes involved in the regulation of apoptosis, particularly the FAS gene. Patients with ALPS often present with recurrent infections due to immune dysregulation, autoimmune cytopenias, and an increased risk of lymphoproliferative disorders, including lymphoma. The condition typically manifests in childhood, but it can also present in adulthood. Diagnosis is based on clinical findings, family history, and laboratory tests that may include lymphocyte subset analysis and genetic testing. Management focuses on symptomatic treatment, including immunosuppressive therapy for autoimmune complications and monitoring for malignancies. Due to the complexity of the immune system's involvement, ALPS requires a multidisciplinary approach for optimal patient care.
Detailed clinical notes on lymphocyte counts, autoimmune manifestations, and treatment responses.
Patients presenting with unexplained lymphadenopathy or splenomegaly, recurrent infections, or autoimmune cytopenias.
Ensure comprehensive documentation of all hematologic parameters and any associated autoimmune conditions.
Thorough immunologic workup results, including lymphocyte subset analysis and autoimmune serologies.
Patients with recurrent infections and autoimmune symptoms requiring immunologic evaluation.
Document genetic testing results and family history of autoimmune diseases.
Used to monitor blood counts in patients with ALPS.
Document the reason for CBC, including symptoms and history of autoimmune cytopenias.
Hematology specialists should ensure all relevant lab results are included.
Common symptoms include lymphadenopathy, splenomegaly, recurrent infections, and autoimmune cytopenias such as anemia or thrombocytopenia.
Diagnosis is based on clinical presentation, family history, laboratory findings, and genetic testing for mutations in apoptosis-related genes.
Treatment focuses on managing symptoms and may include immunosuppressive therapy, monitoring for malignancies, and supportive care for autoimmune complications.
