Non-Hodgkin's lymphomas with t(11;14)(q13;q32): a subset of mantle zone/intermediate lymphocytic lymphoma?

We here describe 13 patients with non-Hodgkin's lymphoma (NHL) and a translocation t(11:14)(q13:q32). They were part of a series of 163 patients with NHL and an abnormal karyotype, serially referred to our institution between January 1984 and 1990. Patients with t(11:14) seem to present several common and interesting features. Males are more frequently affected than females, and old people more than young. They present at diagnosis with advanced disease and usually show involvement of epithelium and bone marrow. With respect to histologic diagnoses, these patients are usually considered to be of low-grade malignancies. However, most of them do very poorly, have short complete remission and frequent relapses whatever the treatment. As a whole, the median survival rate is rather low. The cytologic, histologic as well as the immunologic patterns tend to be uniform: tumours are composed of small cells and display features of mantle zone/intermediate lymphocytic lymphoma. They express high IgM and low IgD levels and more commonly bear Ig lambda light chains. They also express all pan-B antigens (except CD23) as well as the CD5 antigen, but usually lack the CD10. According to these characteristics, these tumours could be placed in between lymphocytic lymphomas (which usually express CD23) and follicular lymphomas (which commonly lack IgD and CD5 and bear CD10 as well as a t(14:18).     

Antithrombin and protein C in systemic inflammatory response syndrome

Coronary artery bypass grafting with cardiopulmonary bypass can induce systemic inflammatory response syndrome. To assess the prevalence of preoperative antithrombin and protein C deficiencies in relation to the incidence of this syndrome, antithrombin and protein C levels were measured in 130 patients undergoing coronary artery bypass grafting with cardiopulmonary bypass. Systemic inflammatory response syndrome developed in 36 (27.7%) patients who were predominantly male, had a lower EuroSCORE, longer cardiopulmonary bypass time, higher pre-bypass temperature, and shorter activated coagulation time. Logistic regression showed that predictive factors included bypass duration and pre-bypass temperature; however, low antithrombin levels appeared to be a negative predictive factor. Antithrombin levels were < 80% in 33.8% of patients, and 11.6% had protein C levels < 80%. Postoperative antithrombin and protein C deficiencies are not uncommon in adults undergoing cardiac surgery with cardiopulmonary bypass, but detection of these deficits did not identify patients at increased risk of systemic inflammatory response syndrome.     

Prevalence of hepatitis C virus infection in asymptomatic anti-HIV1 negative pregnant women and their children

The prevalence of hepatitis C virus (HCV) infection was studied prospectively in pregnant women in France and their children by detection of anti-HCV with second-generation ELISA (ELISA2). In ELISA2-positive women, anti-HCV was detected with second- and third-generation RIBA (RIBA2 and RIBA3) and serum HCV RNA was detected with PCR. Among 670 women, anti-HIV1-negative, 26 (3.9%) were positive with ELISA2. RIBA2 was positive in 13 and HCV RNA was found in 10. Ten ELISA2-positive women had a further evaluation with assessment of HCV infection in their children. Among the 10 children born to the index pregnancy, only one was positive with ELISA2 and RIBA2 but negative with RIBA3 and PCR; the nine other children were ELISA2, RIBA2, RIBA3, and PCR negative. All 26 siblings (2–16 years old), of whom 14 were born to PCR-positive mothers, were ELISA2 and RIBA2 negative. We conclude that among anti-HIV1-negative pregnant women with normal serum ALT levels, the prevalence of HCV infection is relatively high but the risk for mother-to-infant transmission of HCV seems to be low.     

Nonuniformity of the transmural distribution of coronary blood flow during the cardiac cycle. In vivo documentation by contrast echocardiography

This study was performed to examine the transmural (endocardial vs. epicardial) heterogeneity of myocardial blood flow during the cardiac cycle (systole vs. diastole). Twenty-four contrast echocardiographic injections were performed in seven open-chest anesthetized dogs either into left anterior descending or circumflex coronary artery or into the aortic root. Two-dimensional echocardiography in short-axis view was performed and was digitized off-line into a 256 x 256 pixel matrix with 256 gray levels/pixel. All end-diastolic and end-systolic frames before and to peak contrast were analyzed. A region of interest corresponding to the most intensely opacified myocardial segment was traced, the mean videodensity measured, and the frame of initial contrast appearance detected. The region of interest was divided into three equal parallel layers corresponding to the endocardial, midcardial, and epicardial myocardium. When the echocardiographic contrast effect initially appeared in diastole, the increment in videodensity was greater for the endocardium (131 +/- 48%) than for the epicardium (71 +/- 37% of the increment in videodensity of the entire wall) (p less than 0.05). This inhomogeneity subsequently disappeared in the following end-systolic frame. When the initial echocardiographic contrast effect appeared in systole, intensity was higher in epicardium (136 +/- 83%) than in endocardium (60 +/- 60%) (p less than 0.05). However, in the following diastole, intensity was not significantly different for the two layers. Thus, myocardial contrast echocardiography demonstrates that coronary blood flow is primarily subendocardial in distribution during diastole and subepicardial during systole.     

Late haematological complications in severe aplastic anaemia

137 patients with severe aplastic anaemia (SAA) were treated in Basel from 1976 to 1986. 34 underwent bone marrow transplantation (BMT) and 103 received antilymphocyte globulin (ALG) therapy. We have analysed the incidence of late haematological complications in both groups of patients. 20 patients treated with ALG developed a late haematological complication. A myelodysplastic syndrome or frank leukaemia occurred in eight and paroxysmal nocturnal haemoglobinuria (PNH) in 13 patients. Nine of the 13 patients with PNH had clinical signs of haemolysis, four only had positive laboratory tests. One patient had PNH and acute leukaemia. The risk of developing a haematological complication increased continuously and reached 57% at 8 years. Neither PNH nor leukaemia occurred in patients treated with BMT. The increased survival rate and the long observation time after ALG therapy have revealed a new perspective of the prognosis of aplastic anaemia. Patients treated with BMT appear to be cured whereas those treated with ALG remain at risk for late complications.     

Quality-adjusted survival analysis shows differences in outcome after immunosuppression or bone marrow transplantation in aplastic anemia

Bone marrow transplantation (BMT) and immunosuppression (IS) have improved the prognosis of aplastic anemia; both treatments have specific advantages and drawbacks but similar survival rates. Analysis of additional endpoints may help in treatment decisions. In a single-center study, patients with aplastic anemia treated with IS (n=155) or BMT (n=52) were compared for survival, event-free survival, and quality-adjusted time without symptoms and toxicity (Q-TWiST). Probability of overall and event-free survival at 15 years was similar among both groups (BMT 51±15% and 25±14%, IS 53±10% and 27±8%), with more early deaths in the transplant group and more late deaths in the IS group. There were differences in terms of mean duration of seven analyzed health states: time with symptoms from treatment-related toxicity (IS 0.36 years, BMT 0.27), transfusion dependency (IS 0.66 years, BMT 0.1 years), partial remission (IS 3.27 years, BMT 1.42), and secondary clonal disorder (IS 0.68 years, BMT 0.04) was significantly longer for IS compared to BMT (p0.001). Patients treated with BMT spent more time with extensive chronic graft-versus-host disease (GvHD) (IS 0 years, BMT 0.96, p<0.023) and in CR without drugs (IS 1.22 years, BMT 2.43, p=0.056). In conclusion, survival, event-free survival, and Q-TWiST are similar. BMT-treated patients had longer periods free from symptoms, while IS-treated patients needed closer medical care, transfusion support, and medications.