MHC class II deficiency

A severe combined immunodeficiency that is characterized by deficiency of MHC class II molecules that causes lack of immune protection against bacteria, viruses, and fungi and thus causes early death in childhood, and has_material_basis_in autosomal recessive

A severe combined immunodeficiency that is characterized by deficiency of MHC class II molecules that causes lack of immune protection against bacteria, viruses, and fungi and thus causes early death in childhood, and has_material_basis_in autosomal recessive inheritance of mutation in the CIITA, RFX5, RFXANK, and RFXAP genes.

Signs and symptoms

• Recurrent urinary tract infections
• Malabsorption
• Recurrent lower respiratory tract infections
• Agammaglobulinemia
• Colitis
• Protracted diarrhea
• Recurrent protozoan infections
• Infectious encephalitis
• Biliary tract abnormality
• Chronic lymphocytic meningitis

Medications that may treat it

elapegademase pegademase bovine

Also known as: BLSII; SCID due to absent class II HLA antigens; bare lymphocyte syndrome type II