Changes in the activity and transcription of UDP-N-acetylglucosamine: beta-D-mannoside beta-1,4-N-acetylglucosaminyl-transferase III (GnT-III: EC 2.4.1.144) were investigated in haematological malignancies. GnT-III activity was elevated in patients with chronic myelogeneous leukaemia in blast crisis (CML-BC) and patients with multiple myeloma (MM); whereas most of the normal healthy subjects and patients with other haematological malignancies, including CML in its chronic phase, showed negligible activity. The GnT-III transcript of leukaemic cells from various haematological diseases showed a single band with a similar size. The ratio of GnT-III activity per normalized transcript in CML-BC was considerably higher than in the other conditions, which provided the possibility that in CML-BC the transcript or the enzyme protein might be more stable, or that a post-translational modification of the enzyme might enhance its activity. Furthermore, a lectin blot analysis of patient specimens and a lectin fluorescence study of CML cell lines revealed that E4-PHA binding to surface glycoproteins correlated with GnT-III activity, indicating that more bisecting GlcNAc was added to these glycoproteins, catalysed by elevated GnT-III in CML-BC.