Typical and atypical chronic lymphocytic leukemia differ clinically and immunophenotypically.

We compared the features of 17 cases of atypical chronic lymphocytic leukemia (aCLL) with those of a clinical control group of 24 cases of CLL. Quantitative flow cytometric data, available for 12 cases, were compared with an immunophenotypic control group of 58 cases using a relative fluorescence indexfor CD5, CD23, CD79b, and surface immunoglobulin light chain (sIg). Compared with the clinical control group, patients with aCLL had a higher mean WBC count and a lower platelet count. Patients with aCLL had a significantly higher probability of disease progression. Compared with an immunophenotypic control group of 58 CLL cases, 12 cases of aCLL demonstrated significantly higher expression of CD23. There was no significant difference in expression of sIg, CD79b, or CD5 between the groups. CD38 expression was noted in only 1 (9%) of 11 tested cases; 2 (18%) of 11 cases had trisomy 12. aCLL can be distinguished from typical CLL morphologically, clinically, and immunophenotypically. Atypical morphologic features in CLL seem to be a marker of aggressive clinical behavior.

Usefulness of CD79b expression in the diagnosis of B-cell chronic lymphoproliferative disorders.

We evaluated anti-CD79b for its usefulness in the diagnosis of B-cell chronic lymphoproliferative disorders (BCLPDs), particularly chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL). We analyzed 100 BCLPDs for CD5, CD19, CD20, CD23, CD79b, and surface immunoglobulin light chain (sIg) expression by 4-color flow cytometry. CD20, CD79b, and sIg expression were quantified. Correlational analysis and univariable and multivariable logistic regression models were used to determine the best combination of antigens for the immunophenotypic classification of CLL vs other BCLPDs. Positive and statistically significant Spearman pairwise correlations between CD20, CD79b, and sIg fluorescence intensity were demonstrated. In the simplest models in which a single variable was considered, cutoff points were chosen that gave misclassification rates for CLL of 16% for CD79b, 19% for sIg, and 18% for CD20. Low-intensity CD79b, CD20, and sIg are associated highly with CLL. A panel containing CD5, CD19, CD23, and sIg allowed correct classification of most cases. Addition of CD20 or CD79b improved diagnostic accuracy; CD79b was slightly better than CD20. CD79b seems to be a useful addition to a standard flow cytometry panel for the evaluation of BCLPDs.

Transitional portfolios: orchestrating our professional competence.

Reflective professional development results from thoughtful planning of goals and meeting learning objectives. With rapid changes in health care resulting in major reevaluation and even modification of the role and functions of an occupational therapy practitioner, producing a historical record of competencies in order to learn from one's own experience and to plan for change is essential. A new use of the portfolio is presented to show that a portfolio can be more than the typical repository of completed projects. The transitional portfolio is introduced as a tool for one to self-direct professional development through thoughtful engagement with artifacts created during one's career in relationship to professional goals and desired roles. The transitional portfolio process is holistic, flexible, and reflective. It can serve as a way to document accumulated knowledge and skill acquisition as well as facilitate thoughtful, planned reflection on one's career development in occupational therapy. This article describes the use of transitional portfolios to connect professional development learning experiences, accomplishments, and future opportunities for occupational therapy practitioners.

The neuropathology of orthotopic liver transplantation: an autopsy series of 16 patients.

OBJECTIVE: To examine the neuropathologic findings seen in the setting of orthotopic liver transplantation (OLT) and to asses the role, if any, that the neuropathology had in the patient's death. DESIGN: Retrospective autopsy series of 16 patients. SETTING: Tertiary referral center with a high volume of liver transplantation. PATIENTS: Sixteen OLT patients who died and in whom a complete autopsy, including examination of the brain and spinal cord, was performed. RESULTS: Sixteen patients, including 13 women and 3 men, comprised the study group. Patients ranged in age from 25 to 64 years (mean 44.8 years). Postoperative OLT survival ranged from 1 to 1962 days (mean 236 days). Reasons for the initial OLT included hepatitis (n = 6), fulminant hepatic failure (n = 4), cryptogenic cirrhosis (n = 2), methotrexate toxicity (n = 1), postoperative complication (n = 1), primary biliary cirrhosis (n = 1), and hepatocellular carcinoma (n = 1). Autopsies in 13 (81%) patients showed neuropathology; in only 2 patients, however, was the primary cause of death attributable to these findings. The most common neuropathology was related to anoxia or infarction, specifically, ischemia or focal neuronal necrosis (n = 9), infarction (n = 4), and diffuse anoxic encephalopathy (n = 3). Other central nervous system findings included infection with Aspergillus, Candida, and Toxoplasma (n = 3). The most common cause of death was infection-related in 8 patients. One patient died of pulmonary hypertension, 1 of acute rejection, and 1 of possible hyperacute rejection. Two patients died directly as a consequence of neuropathology findings; one had massive central edema with herniation, and the other had a large intracerebral hemorrhage with herniation. The exact cause of death was unclear in 3 patients. CONCLUSIONS: The most common neuropathology findings in this series were related to ischemia and infarction. Neuropathology findings are a significant cause of morbidity, but were only rarely the main cause of death (n = 2) in the OLT patients in this study.

Plexiform neurofibroma with and without associated malignant peripheral nerve sheath tumor: a clinicopathologic and immunohistochemical analysis of 54 cases.

Plexiform neurofibroma (PNF) is an important part of the diagnostic criteria for neurofibromatosis type 1 (NF1) and is a known precursor lesion of malignant peripheral nerve sheath tumor (MPNST). We studied the clinicopathologic features of 54 cases of PNF for which the hematoxylin- and eosin-stained slides and paraffin blocks were available and adequate clinical follow-up could be obtained. In addition, in all cases, a representative section of the PNF and, when present, MPNST, was evaluated immunohistochemically with an antibody for p53 (DO7). The cohort included 28 male patients and 26 female patients, with an age range from 4 to 79 years (mean, 27 yr). Of these 54 patients, 46 (85%) met the strict diagnostic criteria for NF1. Thirty-nine patients had PNF alone; 15 patients had an MPNST arising from the PNF (PNF/MPNST). Those patients with PNF/MPNST tended to be older (38 yr vs. 22 yr) and to have larger tumors (10.5 cm mean vs. 7.4 cm mean) than those with PNF alone. In 9 patients (23%) of 39 with PNF alone, local recurrence developed, whereas in 7 patients (47%) of 15 with PNF/MPNST, recurrent MPNST developed, and metastases developed in 3 (20%) of the 15. Immunohistochemically, only 1 case (2.5%) of 39 cases of PNF alone stained for p53. On the other hand, 12 (80%) of 15 cases of PNF/MPNST showed p53 immunoreactivity in the MPNST component, 2 of which also showed staining in the PNF areas. In conclusion, we found that the vast majority of patients with PNF met the strict diagnostic criteria for NF1. The immunohistochemical detection of intranuclear p53 protein is common in the malignant areas of PNF/MPNST but is rare in the PNF regions. The rarity of p53 staining in the PNF regions precludes its use in predicting those tumors that are likely to progress to MPNST.

Paraneoplastic pemphigus occurring in a patient with B-cell non-Hodgkin's lymphoma.

We present the case of a 71-year-old white male with paraneoplastic pemphigus associated with a B-cell non-Hodgkin's lymphoma. Diagnosis of paraneoplastic pemphigus was made by the characteristic findings on immunoprecipitation performed on a serum specimen. Paraneoplastic pemphigus is a severe autoimmune disease comprised of polymorphous mucocutaneous lesions, characteristic laboratory findings, association with one of several types of neoplasms, and a very poor prognosis.

Littoral cell angioma of the spleen.

Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen. LCA most commonly presents with constitutional symptoms (low grade fever and fatigue) and signs of hypersplenism (anemia and thrombocytopenia). Radiographically and at gross pathology an enlarged spleen containing multiple nodules is most commonly seen. Currently the radiological findings are nonspecific and correlation with clinical findings is necessary to narrow the differential while tissue is required for a definitive diagnosis.

Theory-based practice in the workplace: the next step.

In this article, an educator and a clinician address issues involved in implementing a course that helped rehabilitation nurses forge the critical link between theory and practice. A combination of strategies was used to present the material in a three-session inservice program.

Cognitive dysfunction following cardiac arrest.

Recent work that has been done in cardiopulmonary cerebral resuscitation has focused on the area of brain resuscitation and the prevention of neurologic deficits. The Glascow Coma Scale and Glascow-Pittsburgh Scale are useful clinical tools in the acute stages of survival; however, they fail to provide sufficient data relative to cognitive disturbances. The Levels of Cognitive Functioning Scale applied to the postcardiac arrest patient may be helpful in guiding therapeutic nursing interventions and nursing research in the Critical Care environment.

Antiseptal brain antibody in IgG of schizophrenic patients.

On the hypothesis that schizophrenia is an immunological disorder in which antibody is produced against a unique antigen sequestered principally or exclusively in the septal region of the brain, we used crossed-immunoelectrophoresis (CIE) to evaluate reactivity of a gamma G immunoglobulin (IgG) fraction from serum of schizophrenic patients and nonschizophrenic control subjects with homogenates of tissues of septal region, hippocampus, vermal cerebellum, frontal cortex, and liver of rhesus monkeys. When IgG fractions of unmedicated schizophrenic patients and schizophrenic patients who had received neuroleptic medication for less than 24 hr were tested against septal region homogenate, a precipitin arc was identified, indicating a positive result, with more than 95% of the fractions. In contrast, IgG fractions of schizophrenic patients who had received neuroleptic medication for more than 24 hr were rarely positive. When schizophrenic fractions that tested positive against septal region homogenate were tested against homogenates of the other tissues, they were negative. Fractions of all nonschizophrenic control subjects were negative against all homogenates.

Urobehavioral intervention in the rehabilitation of lower urinary tract dysfunction: a case report.

A case study illustrates the usefulness of behavioral analysis and paradoxical strategy in the treatment of life-long urgency incontinence in a cerebral palsy patient. Volitional increase in diurnal enuresis appeared to result in rapid (within 5 days) acquisition of continence. From pretreatment to 13 month follow-up interview, the weekly wetting frequency decreased from an average of 25 to 0.5 episodes. Symptom rehearsal is believed to have interrupted the typical pathobehavioral sequence between the urge to void and micturition.

Adjuvant properties of Micropolyspora faeni.

The adjuvant properties of Micropolyspora faeni, an important source of antigenic material in the production of farmer's lung, were evaluated by comparing antibody- and cell-mediated immune responses of rabbits to bovine serum albumin (BSA) incorporated in complete Freund's adjuvant (CFA), incomplete Freund's adjuvant (IFA) and incomplete Freund's adjuvant with 5-10 mg/ml homogenized M. faeni (MFA). Rabbits immunized with BSA in CFA or MFA developed significantly increased antigen-induced macrophage migration inhibition, lymphocyte stimulation, and delayed skin reactivity when compared to those immunized with BSA in IFA. No similar adjuvant effect on specific antibody production was observed in rabbits immunized using BSA in MFA. These data suggest that M. faeni can act as a selective immunologic adjuvant for delayed hypersensitivity. This adjuvant property might be important in the induction of mononuclear cell infiltrates seen in human hypersensitivity pneumonitis.