Systemic mast cell disease (SMCD) can be regarded as a tumorous prolif
eration of tissue mast cells involving various organs. The frequency w
ith which SMCD is found in patients with haematological disorders sugg
ests that the association is non-random. The association includes prim
arily, myeloid disorders such as myelodysplastic syndromes and acute o
r chronic myeloproliferative disorders. Lymphoproliferative disorders
may also occur but more rarely, mostly non-Hodgkin's low grade B cell
lymphomas. In this report a case is described in which SMCD occurred i
n a patient with hairy cell leukaemia.