目的　确定原发性旁路高胆红素血症为遗传病并确定其遗传方式。方法　临床病例结合家系谱分析。结果　原发性旁路高胆红素血症患者除表现为黄疸、贫血和脾大外 ,尚有外周血白细胞数减少 ,网织红细胞数不升高 ,外周血和骨髓成熟红细胞形态异常和大小不一 ,可见靶形 ,杆状 ,口形和球形等异常红细胞。家系谱分析发现 ,4代人每代都有患者 ,垂直传播 ,男女均患病 ,成人显性充分 ,病情严重程度不一。结论　原发性旁路高胆红素血症是一种遗传性疾病 ,遗传方式为常染色体显性遗传。建议将原发性旁路高胆红素血症更名为遗传性旁路高胆红素血症 Objective To determine whether primary bypass hyperbilirubinemia is a genetic disease and to determine its genetic pattern. Methods Clinical cases combined pedigree analysis. Results In addition to the performance of primary bypass hyperbilirubinemia in patients with jaundice, anemia and splenomegaly, there are still fewer peripheral blood leukocytes, reticulocytes does not increase the number of peripheral blood and bone marrow mature erythrocytes abnormalities and size Different, visible target-shaped, rod-shaped, mouth-shaped and spherical abnormalities such as red blood cells. Pedigree analysis found that each generation of 4 generations of patients with vertical transmission, both men and women are sick, adult dominance, the severity of the disease varies. Conclusions Primary bypass hyperbilirubinemia is a hereditary disease characterized by autosomal dominant inheritance. Proposed primary bypass hyperbilirubinemia was renamed hereditary bypass hyperbilirubinemia