In this study, we (1) elucidated the prevalence of HL caused by TECTA mutations in Japanese ADSNHL patients, (2) examined the genotype-phenotype correlations for each domain, (3) analyzed the rate of HL deterioration for TECTA-associated HL patients, and (4) carried out haplotype analysis for one variant identified in four unrelated ADSNHL families to confirm whether the mutation occurred in a mutational hotspot or whether it was a founder mutation. The gene discussed is TECTA; the disease is Hodgkins lymphoma.