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A Cuban family with a new congenital dysprothrombinaemia is described. The propositus was a 5-year-old female who presented with umbilical bleeding after birth followed by easy bruising and bleeding tendency throughout her life. The main laboratory features of the defect included prolongation of prothrombin time and partial thromboplastin time. Prothrombin activity was less than 10% in several one- and two-stage systems. However, the staphylocoagulase-complexed prothrombin level and immunologic methods yielded levels of about 50%. The migration of the abnormal prothrombin was more anodic in single and bidimensional immunoelectrophoresis system and did not change by the addition of calcium. Family studies revealed that the father had approximately 50% prothrombin activity and antigen, whereas the mother had 45% prothrombin activity but about 100% prothrombin antigen. We suggest that the propositus is heterozygous for an abnormal prothrombin and heterozygous for true prothrombin deficiency.

Summary. In a patient with a history of anaemia, jaundice and splenomegaly, the bone marrow showed erythropoietic hyperplasia with 20% of multinucleated erythroblasts. His red cells gave high agglutination scores with anti‐i, and haemolysis tests with acidified iso‐group sera were positive. A type II congenital dyserythropoietic anaemia was diagnosed in this patient and this was shown to be associated with von Willebrand's disease. A brother of the propositus also had a positive acidified serum test and showed agglutination by anti‐i, but polynucleated erythroblasts were not found.