THIAMINE-RESPONSIVE MYELODYSPLASIA

The triad of thiamine-responsive anaemia, diabetes mellitus and deafne ss has been reported in 15 patients with macrocytic anaemia, sometimes associated with moderate thrombocytopenia, The bone marrow aspirate u sually shows megaloblastic changes and ringed sideroblasts, However, t ri-lineage myelodysplasia has never been reported, We describe two pat ients who presented with diabetes, deafness and thiamine-responsive pa ncytopenia. Bone marrow aspirate and biopsy were typical of tri-lineag e myelodysplasia. These Endings suggest that thiamine may have a role in the regulation of haemopoiesis at the stem cell level, We propose t he term 'thiamine-responsive myelodysplasia' rather than that of thiam ine-responsive anaemia.