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 The distinction from lymphoblastic leukaemia and from CLL w a s originally made on the basis of the peculiar morphology of the lymphocytes as seen in Romanowsky-stained fiims of peripheral blood and bone marrow.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
Disease stage and recipient/donor human leukocyte antigen (HLA) matching are important determinants of outcome in transplantation using volunteer-unrelated donors (VUD). Matching for HLA-A, -B, -C, -DRB1, -DQB1 is beneficial, whereas the importance of DPB1 matching is more controversial. The impact of HLA matching status may differ dependent on disease stage. We investigated the outcome according to the degree of HLA matching at 6 loci, in 488 recipients of predominantly T-cell depleted bone marrow VUD transplants for leukaemia. Survival was significantly better in 12/12-matched transplants in those with early leukaemia (5 years: 63 versus 41% in 10/10 matched, P=0.006), but not late stage disease. Conversely, within the HLA-mismatched group (< or =9/10), there was a significant survival advantage to DPB1 mismatching (5 years: 39 versus 21% in DPB1 matched, P=0.008), particularly in late leukaemia (P=0.01), persisting in multivariate analysis (odds ratio 0.478; 95% confidence interval 0.30, 0.75; P=0.001). These novel findings suggest that the best outcome for patients with early leukaemia, with a 10/10-matched donor, is achieved by matching for DPB1. Conversely, our results suggest that in patients receiving an HLA-mismatched graft, the outcome is significantly better if they are also mismatched for DPB1. We recommend validation of these results in independent datasets.
Blood Donor A with asymptomatic acute B19 infection was the source of B19 infection in the bone marrow transplant patient. Donor B with a low level of B19 DNA was not the source of infection.
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