Richter's syndrome with different immunoglobulin light chains and different heavy chain gene rearrangements

In a patient with Richter's syndrome, the chronic lymphocytic leukemia (CLL) expressed lambda, mu, and delta immunoglobulin (lg) chains and the non-Hodgkin lymphoma (NHL) kappa, mu, and delta lg chains. The difference in lg light chain expression suggests that the CLL and NHL are independent malignancies, or that the oncogenic event occurred in a B cell differentiation stage after the heavy chain gene rearrangements but before the selection of the light chain. Analysis of DNA by Southern blotting revealed that the lg heavy chain genes of the two malignancies were rearranged in a different way. We therefore conclude that in this patient the NHL cannot be regarded as a progression of the CLL but should most likely be considered as an independent B cell malignancy, which arose in a susceptible host.     

Autoantibodies against bactericidal/permeability-increasing protein in patients with cystic fibrosis

Cystic fibrosis (CF), a genetic disorder, is characterized by chronic pulmonary infection/inflammation which leads to respiratory failure. The presence of anti-neutrophil cytoplasmic autoantibodies (ANCA) has previously been observed in the sera of patients with CF. In view of the known relationship of ANCA with primary vasculitis and of their putative pathogenetic role in these disorders, we studied the presence, specificity and isotype of ANCA and their clinical associations in 66 adult CF patients. None of the 66 CF samples had autoantibodies to the major ANCA antigens, proteinase 3 or myeloperoxidase. However, 60/66 (91%) CF samples contained IgG and 55/66 (83%) IgA, autoantibodies to bactericidal/permeability increasing protein (BPI), a recently characterized ANCA specificity. All the IgA anti-BPI-positive samples were also IgG anti-BPI-positive. The autoantibody specificity was confirmed by inhibition assay and immunoblotting of CF sera against a neutrophil granule preparation. Furthermore, in this cross-sectional study, anti-BPI levels were inversely correlated with the observed reductions in FEV1 and FVC (IgA anti-BPI and FEV1: r = 0.508, <it>p</it> &lt; 0.0001), and both IgG and IgA anti-BPI levels were higher in CF patients with secondary vasculitis (<it>n</it> = 6) than in those without (<it>p</it> &lt; 0.05). ANCA with specificity for BPI were present in the majority of CF sera in this study and autoimmune processes may be associated with the development of pulmonary injury in CF.      

The effect of cuff pressure deflation rate on accuracy in indirect measurement of blood pressure with the auscultatory method

The importance of cuff deflation rate in the auscultatory method of measuring blood pressure was investigated using a computer-based model. To determine the relationship between the cuff deflation rate and the measurement error, two cuff deflation protocols were used, one based on heart rate (mm Hg per heartbeat), the other on a constant rate (mm Hg per second). The different deflation protocols and rates were tested using a constant blood pressure of 120/80 mm Hg and heart rates ranging from 40 to 120 beats/min. It was confirmed that a cuff deflation rate that is time based will introduce larger errors at low heart rates. Using heart rate as a basis for cuff deflation rate yields a constant error that is independent of heart rate. The currently used standard of 3 mm Hg/s could result in a maximum error of 2.5 mm Hg in both systolic and diastolic pressures at a heart rate of 72 beats/min. The maximum systolic and diastolic errors increase to more than 4 mm Hg at 40 beats/min. A deflation rate of 2 mm Hg/beat, however, yields a maximum error of 2 mm Hg for both systolic and diastolic pressures, independent of heart rate. A cuff deflation rate based on heart rate is recommended to help minimize changes in measurement error when measuring blood pressure if a wide range of heart rates will be encountered.Supported by grants from IVAC, San Diego, CA, and Physio Control, Redmond, WA.     

Factors influencing capnography in the bain circuit

The Bain circuit provides continuous fresh gas flow near the airway. The potential mixing of this fresh gas with expired gas may prevent reliable analysis of expired gas. We therefore investigated the interaction of sampling site, fresh gas flow rate, expiratory flow rate, and sampling flow rate on expiratory capnography. Sampling near the fresh gas outlet yielded inaccurate results under several of these conditions. The magnitude of the error was related to the fresh gas and expiratory flow rates. A reliable sampling region near the endotracheal tube was identified.     

Cardiovascular Disease in Early Kidney Transplantation: Comparison Between Living and Deceased Donor Recipients

BackgroundCardiovascular disease (CVD) mortality is extremely high among kidney transplant recipients (KTRs), particularly in the first months after transplantation. Few data are available comparing the cardiovascular profile between KTRs from living versus deceased donors.Objectives and methodsThe aim of the present study was to evaluate the prevalence of CVD in the first 2 months following transplantation, among 120 KTRs of living versus deceased donor organs.ResultsLeft ventricular hypertrophy was observed in 65% of patients, coronary artery calcification in 30%, and cardiac arrhythmias in 46%. CVD was more prevalent among KTRs from deceased versus living donors: ventricular hypertrophy 87% versus 59% (P = .008); coronary artery calcification 42% versus 24% (P = .04); and cardiac arrhythmias 59% versus 39% (P = .06). Multiple logistic regression analysis adjusted for age and dialysis vintage, showed graft donor to not be associated with the prevalence of any CVD (β coefficient 0.912, 95% confidence interval 0.276–3.012, P = .88).ConclusionIn conclusion, the present study demonstrated an elevated prevalence of CVD among KTRs. Patient characteristics, mainly longer length on dialysis seemed to contribute to a greater prevalence of cardiovascular complications among KTRs from deceased compared with living donors on univariate but not multivariate analysis.     

Genetic control of specific immune suppression. IV. Responsiveness to the random copolymer L-glutamic acid(50)-L-tyrosine(50) induced in BALB/c mice by cyclophosphamide

Previous reports from our laboratory have demonstrated the stimulation of specific suppressor T cells in genetic nonresponder mice after immunization with the terpolymer of L- glutamic acid, L-alanine, and L-tyrosine (GAT) (1,2) and with the copolymer of L-glutamic acid and L-tyrosine (GT) (3-5). These findings raise two important questions: (a) do the specific suppressor T cells inhibit an antibody response which would otherwise develop in nonresponder mice; and, (b) can specific helper T cells inhibit an antibody response which would otherwise develop in nonresponder mice; and, (b) can specific helper T-cell activity be detected in these animals. Responsiveness appears to be completely dominant over suppression in (responder x suppressor)F(1) hybrids, therefore, we have been unable to detect suppressor cells in these hybrids after conventional immunization with GAT (2). However , using special conditions of antigen administration, GAT helper activity could be demonstrated in nonresponder DBA/1 (“suppressor”) mice. Thus, GAT-specific helper activity was not detected in these nonresponder animals after immunization with GAT irrespective of the adjuvant used, but could be stimulated by macrophage-bound GAT or by GAT complexed with methylated bovine serum albumin GAT-MBSA (6). In the current report we have taken advantage of the fact that suppressor T-cell activity is more sensitive to cyclophosphamide treatment than T-cell helper activity (7) to demonstrate the presence of GT-specific helper activity in “nonresponder” BALB/c mice. We describe: (a) the dose of cyclophosphamide and conditions of treatment which inhibits the well-documented stimulation of specific suppressor T cells in BALB/c mice injected with GT previous to immunization with GT-MBSA, and (b) the ability of cyclophosphamide to permit the development of primary PFC responses to GT in these “nonresponder” mice. These cyclophosphamide-induced responses are not characterized by the high levels of antibody detected in genetic responder animals.