The clinical and haematological features of 15 patients with a rare variant of chronic 1 ymphocytic leukaemia (CLL) are described. The disease predominantly affects males in the sixth and seventh decades of life and presenting symptoms include fatigue, weakness, weight loss, sweats and fevers. Massive enlargement of thc spleen (mean weight at autopsy 1383 g, range 227-3500 g) and to a lesser extent of the liver (mean weight 2445 g, range 2030-307g g) are regular findings. In contrast, peripheral lymphadenopathy is inconspicuous or absent. The characteristic cell in the peripheral blood is a relatively large lymphoid cell w i t h a large vesicular nucleolus, relatively well-condensed nuclear chromatin and moderate amount of cytoplasm. The counts of these cells in the peripheral blood at the time of diagnosis are very high (mean 355 ooo/~l, range 26 000-1 I I O w/~l). The clinical response to methods of treatment that are usually effective in classical CLL (particularly alkylating agents and corticosteroid drugs) is uniformly poor and the patients' survival after diagnosis is in most cases quite short.We believe that the clinical and haematological features of this condition justify its recognition separately from classical CLL, from lymphosarcoma cell leukaemia and from acute lymphoblastic leukaemia. We have therefore designated it 'prolymphocytic leukaemia'. On the basis of a single case we suggest that further trials of splenectomy are indicated.Since 1956 we have encountered 15 patients suffering from a type of lymphocytic leukaemia with several characteristics that distinguish it from classical chronic lymphocytic leukaeniia (CLL). The patients were referred to us as cases of acute lymphoblastic leukaemia, CLL, or in one case as myeloblastic leukaemia. The distinction from lymphoblastic leukaemia and from CLL w a s originally made on the basis of the peculiar morphology of the lymphocytes as seen in Romanowsky-stained fiims of peripheral blood and bone marrow. Later it became apparent that in addition to the lymphocyte morphology these patients all had certain other features in common. In general the presenting symptoms were of short duration, the principal physical signs were gross splenic and slight to gross hepatic enlargement with little or no enlargement of lymph nodes, the absolute lymphocyte counts in the peripheral blood were G.J.G. is now Associate Professor

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The long terminal repeat (LTR) of the AIDS virus (HIV) has been found to contain promoter sequences that are active in uninfected HeLa whole cell and nuclear extracts. Here we report that elements upstream of position -104 (start site +1) do not affect transcriptional activity in vitro whereas sequences between -104 and -57 are required for such activity. Using a reconstituted RNA polymerase II system, we demonstrate that a partially purified fraction containing Spl not only stimulates, as was previously reported, but is required for accurate initiation of transcription directed by the HIV LTR. In addition, based on a computerized analysis, we report the presence of a region in the HIV LTR (positions -151 to -80) that is similar to the 72 base pair enhancer element of SV40 and that includes a highly conserved segment also present in the cytomegalovirus enhancer. Moreover, the HIV and HTLV-I LTRs are shown to share a region of similarity that includes the 21 base pair motif found in the enhancers of the human and bovine T-lymphotropic viruses. The R region of the HIV LTR is found to have two extensive regions of dyad symmetry rather than one as was previously reported. The significance of these observations for HIV pathogenesis is discussed.