LIGHD describes the third patient with autosomal recessive BCL10 complete deficiency. This patient has a nonsense mutation that leads to a loss of expression (K63X). Results are published in Frontiers in Immunology in a report entitled: Clinical and immunological features of human BCL10 deficiency. This report highlights the importance of early genetic diagnosis for the management of BCL10 deficient patients and HSCT as the recommended treatment to cured this disease.