Acquired Haemoglobin H Disease, Complicating a Myeloproliferative Syndrome: A Case Report

Summary A case of acquired haemoglobin H disease in association with a myeloproliferative disorder is described. Severe haemolysis with hypochromic microcytic anaemia was present. Haemoglobin H formed 18% of the circulating haemoglobin and 60% of the red cells showed multiple inclusions on incubation with brilliantcresyl blue. Blood film and absolute red cell values from a previous unrelated illness were normal, proving the acquired nature of the haemoglobin abnormality. Alpha/beta chain synthesis was measured in vitro and the degree of imbalance (alpha/beta ratio 0.39) was sirrlilar to that seen in the inborn thalassaemic disorder. A small proportion of red cells showed i-antigen reactivity but their haemoglobin H content was no different from the majority of cells which were I-antigen positive.