A 52-year-old Japanese woman was referred to our hospital because of fever and coxalgia. She had a white blood cell count of 241 Â 10 2 /mL with 59.6% blasts, which had a high nuclear/cytoplasmic ratio and variably condensed nuclear chromatin.Flow cytometry and chromosomal analysis of bone marrow cells indicated positive findings of CD10, CD19, CD34, HLA-DR antigens and t(9; 22)(q34; q11.2), respectively. No rearrangements of bcr/abl in peripheral blood neutrophils were found by fluorescence in situ hybridization, suggesting that she had B-acute lymphoblastic leukaemia with Ph chromosome. Blood glucose and HbA 1c (glycated haemoglobin) levels on admission were 23.4 mmol/L and 21.0%, respectively. The results of 1.5 anhydro-D-glucitol and glycoalbumin tests revealed that she certainly had diabetes mellitus (DM). Insulin therapy was initiated. Her high level of HbA 1c also suggested the possibility that the patient suffered from haemoglobinopathies in addition to DM. Sequencing analyses of a1-, a2-and b-globin genes were all normal. The patient achieved complete remission (CR) by one month after her first course of chemotherapy, and the HbA 1c level decreased to 10.4% following insulin therapy and chemotherapy, which were initiated when she attained CR. Her extremely high HbA 1c level was due mainly to DM. Also, suppression of erythropoiesis by proliferation of leukaemic cells and latent iron deficiency might have partially contributed to the increased HbA 1c . This could result in a transient but extremely high HbA 1c level. To our knowledge, this is the first report of an acute leukaemia patient who expressed an extremely high level of HbA 1c .