Major histocompatibility complex class II deficiency
ICD-10 D81.7 is a billable code used to indicate a diagnosis of major histocompatibility complex class ii deficiency.
Major histocompatibility complex class II (MHC II) deficiency is a rare immunodeficiency disorder characterized by the absence or dysfunction of MHC II molecules on antigen-presenting cells. These molecules are crucial for the activation of CD4+ T lymphocytes, which play a vital role in orchestrating the immune response against pathogens. Patients with MHC II deficiency are particularly susceptible to opportunistic infections, autoimmune diseases, and certain malignancies due to their impaired immune response. The condition can be inherited in an autosomal recessive manner and may present in infancy or early childhood with recurrent infections, failure to thrive, and lymphadenopathy. Diagnosis typically involves genetic testing to identify mutations in genes responsible for MHC II expression, along with immunological assays to assess T-cell function. Management includes supportive care, prophylactic antibiotics, and in some cases, hematopoietic stem cell transplantation to restore immune function.
Detailed immunological assessment and genetic testing results.
Patients presenting with recurrent infections, failure to thrive, or autoimmune symptoms.
Ensure comprehensive documentation of immune function tests and clinical history.
Growth charts, vaccination history, and detailed clinical notes on infections.
Infants or children with unexplained recurrent infections or developmental delays.
Focus on early identification and management of infections and nutritional status.
Used when confirming a diagnosis of MHC II deficiency.
Document the reason for testing and results.
Immunology specialists should ensure comprehensive documentation of clinical findings.
Common symptoms include recurrent infections, failure to thrive, and autoimmune manifestations due to impaired immune response.
