CHRONIC PROLYMPHOCYTOID LEUKEMIA WITH AN UNUSUAL IMMATURE IMMUNOPHENOTYPE

A case of a 58 year old woman with a chronic lymphoproliferative disor der of unusual clinical presentation, disease course, and immunophenot ype is presented. At diagnosis she had severe anaemia, moderate lympho cytosis with some cells having prolymphocytoid features and a normal p latelet count. A clinical examination yielded negative results. Only a naemia related symptoms were found and the patient became blood transf usion dependent. Both the lymphocytosis and the proportion of prolymph ocytoid cells rose insidiously and thrombocytopenia developed later du ring the course of the disease. Three years later, the patient had a w hite cell count of 269 x 10(9)/l almost exclusively of prolymphocytoid cells and the bone marrow was diffusely infiltrated. She was refracto ry to chemotherapy and the anaemia did not improve after treatment wit h cyclosporine. Lymphoid cells were positive for cytoplasmatic CD3, HL A-Dr, CD34, CD38, CD7, CD56, CD13, CD33 and CD65. Membrane alpha beta and gamma delta T cell receptors (TCRs) were not expressed and the bet a chain TCR gene was in germline configuration. Other membrane T, B, n atural killer, and myelomonocytic markers were negative. Karyotype ana lysis was tried several times but metaphases were not obtained, even a fter stimulation with T cell mitogens.