Survivals of two series of CLL patients (99 from a retrospective series and 196 from a prospective series) were studied separately. The three main staging systems (Rai, Binet, Rundles) agreed well, but as far as survival is concerned, too many stages are defined. The authors performed a Cox multivariate analysis of survival in order to isolate important prognostic factors at diagnosis and to use them to build a simple three-stage classification. Thrombopenia and anemia appeared as the most important risk factors. Among the nonanemic and nonthrombopenic patients, the number of involved areas was clearly related to prognosis in the authors' two series. This study allowed the authors to propose a new classification in three prognostic groups. Group C: anemia (Hb less than 10 g) and/or thrombopenia (platelets less than 100,000/mm3); about 15% of the patients; median of 2 years. Group B: no anemia, no thrombopenia, three or more involved areas (counting as one each of the following: axillary, cervical, inguinal, lymph nodes, whether unilateral or bilateral, spleen and liver); about 30% of patients; median of 7 years. Group A: no anemia, no thrombopenia, less than three involved areas; about 55% of patients; the survival of this group does not seem different from that of the French population of the same age and sex distribution. This three-stage classification only requires clinical examination and routine hemogram, has a good prognostic value which was confirmed on the series of Montserrat and Rozman (146 patients), and should therefore be helpful in planning new clinical trials.

One hundred and twenty-nine patients with chronic lymphocytic leukemia (CLL) followed in our outpatient department for periods ranging from 6 months to 13 years were divided into five anatomico-clinical stages: stage 0 (peripheral and bone marrow lymphocytosis); stage I (stage 0 + lymph node enlargement); stage I1 (stage 0 + palpable spleen); stage 111 (stage I + 11); and stage IV (anemia or thrombocytopenia) . Analysis of actuarial survival curves revealed the following: 1) median survival of the entire population exceeded 114 months; 2) there was no difference between the curves of stage 0 and stage I patients, 3) there was a significant difference between survival for stage 111 and IV patients as compared with stages 0, I and I1 (p < 0.01); and 4) median survival for stage I11 and stage IV was 70 months; and 23 months, respectively. Age did not appear to be a prognostic factor. Prognosis was poorer in male patients and in those with a high initial lymphocyte count (50,000/mma), but this was due to the higher incidence of stages 111 and IV in this population ( p <

It has been previously demonstrated that the sea star axial organ is a primitive immune organ. Phagocytic, lymphoid-like cells have been characterized with properties similar to those of vertebrates. There is also evidence for an invertebrate cytokine network because IL-1 and TNF-like activities are clearly demonstrable. In addition, the authors have previously described preliminary evidence for IL-2-like activity in the sea star. In the present report, the authors obtained evidence for the presence of IL-1- and IL-2-like molecules on axial organ cells. More interestingly, the results suggested that sea star cells express structures similar to human receptors for IL-1, IL-2, IL-6 and IFN-gamma.

A case of trisomy 6p21 leads to 6pter resulting from a maternal balanced t(2;6)(p25;p21) translocation is reported. The main clinical abnormalities were psychomotor retardation, hypotrophy, blepharophimosis, nystagmus, high nasal bridge, small mouth, sacral dimple, and systolic murmur. Other anomalies might have been due to partial 2p monosomy. Comparison with seven other cases of trisomy 6p allowed the delineation of a clinical entity. Direct proof of the localization of HLA genes was given by the presence of three haplotypes in the index patient.