Cytogenetic studies of bone marrow and extramedullary tissues and clinical course during metamorphosis of chronic myelocytic leukemia

Of 33 consecutive patients with chronic myelocytic leukemia, examined during metamorphosis, 82% showed chromosome abnormalities in addition to the Ph1. Aberrations most frequently encountered were +8 (39%), +22q - (30%), and i(17q) (18%). Translocations other than the Ph1 were observed in four cases and - Y clones in four cases. Discrepancies in the cytogenetic pattern between bone marrow and extramedullary tissues or blood were noted in a total of 15 patients. In six cases, transformation occurred in extramedullary organs at a time when it was not present in the marrow. In three cases the bone marrow transformation was preceded by a lymph node blastic infiltrate; in one case, by a skin infiltrate; and in one case, by a subdural blastoma. Clonal abnormalities additional to the Ph1 were identified in the tumor tissue from all these cases. Patients with primary extramedullary transformation tended to have a lower median age at onset of metamorphosis, shorter survival, and higher incidence of chromosome abnormalities than the cases without extramedullary involvement. Patients with only Ph1-positive cells and no other anomalies had a slightly longer duration of metamorphosis and longer total survival. Basophilia and thrombocytopenia were more marked in cases with i(17q) than in the rest of the series.     

Different specificities of an HLA-DRw6 haplotype detected by alloreactive T lymphocytes

DRw6 has been difficult to define serologically. In the present experiments we have developed T cell lines in order to characterize the components of a DRw6 haplotype. This was accomplished by priming T cells with allogeneic mononuclear cells mismatched for DRw6, Dw6, and MT2. Subsequently, three sublines with distinct reactivity patterns were derived by limiting dilution. The specificities detected by these sublines included: (a) a specificity found on a subset of cells positive for DRw6 which was inhibited by monoclonal antibodies against DS(DC), the human homologue of the murine IA-encoded molecules, (b) another DRw6-associated specificity blocked by an MT2-like antibody, and (c) an MT2-like specificity blocked by monoclonal antibodies reactive with a different MT2-associated determinant. These results show that more than one IE-like, as well as the DS/DC (IA-like) molecules, carry distinctive antigenic epitopes that can be recognized by allogeneic T cells. Primed T cell lines may be useful for a better definition of certain haplotypes which are at present difficult to characterize with serological reagents alone.     

Bronchocentric granulomatosis: a complication of allergic bronchopulmonary aspergillosis.

Hypersensitivity to the fungal antigens of Aspergillus fumigatus may result in a spectrum of immune injury collectively known as allergic bronchopulmonary aspergillosis (ABPA). This report describes a 14-yr-old boy who presented clinical findings consistent with ABPA,including a history of asthma, blood eosinophilia, serum precipitins, and IgE antibodies to Aspergillus fumigatus. Sputum Aspergillus, pulmonary infiltrates, and dual types I and III skin reactions to Aspergillus fumigatus were observed also. Pathology of the resected right upper lobe revealed severe bronchial destruction with the findings of bronchocentric granulomatosis. Noninvasive septate fungal hyphae compatible with Aspergillus were identified. Cultures from sputum and surgical specimens grew Aspergillus and Mycobacterium intracellulare avium. The PPD-B (purified protein derivative-Batty) intradermal skin test produced a 6 mm induration (PPD-S was negative). The patient's condition has been well controlled with prednisone and several antituberculous drugs. In addition, inflammatory and immunologic parameters have begun to return to normal. The relationship between ABa and the atypical mycobacterial infection is not clear. The association of ABPA with the severe bronchial destruction seen in bronchocentric granulomatosis is emphasized to alert physicans to this serious sequelae of ABa seen in the asthmatic.     

Mediated smoking cessation programs in the Stanford five-city project

Two mediated smoking cessation programs were subjected to a field evaluation. The Quit Kit is a printed self-help package, and "Calling It Quits" consists of five segments which were aired on the local television news. A sample of 239 persons requested the Quit Kit and were followed. At the 2-month and 12-month follow-ups, respectively, 13.6% and 17.9% of those surveyed reported abstinence. Results indicate the potential of mediated interventions.     

Cerebral hemodynamics in patients with moyamoya disease. A study of regional cerebral blood flow by the 133Xe inhalation method

Regional cerebral blood flow was measured by the 133Xe inhalation method in 20 young patients with moyamoya disease and five young healthy volunteers. Most patients showed low values of mean hemispheric blood flow in both hemispheres. Regional cerebral blood flow was at a low value in the upper frontal region and at an almost average value in the posterotemporal and occipital regions, which was different from the "hyperfrontal" pattern in healthy volunteers. Regional cerebral blood flow was reduced evenly by hyperventilation. By 5% CO2 inhalation, regional cerebral blood flow was increased in the temporooccipital regions and was nearly unchanged or decreased in the frontal region.     

Functional inhibition and release: unilateral tachistoscopic performance and stereoelectroencephalographic activity in a case with left limbic status epilepticus

During prolonged nonconvulsive unilateral left limbic status epilepticus, a natural model of functional hemispheric inhibition, we performed two tachistoscopic experiments, a lexical decision task associated with a RVF (left hemisphere) superiority and a facial matching task associated with a LVF (right hemisphere) superiority. We found that epileptic activity in the left hemisphere, especially rhythmic high-frequency "tonic" discharges, inhibited performance on the lexical task but not on the facial matching task. This suggests that only cognitive activity in the discharging hemisphere is inhibited. Strikingly, the best performance of the right hemisphere was obtained while the left hemisphere was most inhibited, suggesting a functional balance of inhibition and release.     

ER "capture" of the skid-row alcoholic

In response to the spiraling anger and frustration voiced by the emergency room (ER) medical staff and the observed negative interactions between the alcohol-dependent patient and the ER staff, the author completed a 6-month retrospective review of all patients evaluated by the ER service with a complaint of alcohol abuse, chronic alcoholism, or requesting detoxification. As a result of that study, an ER-based comprehensive approach to the management of this population was proposed. Utilizing an existing Connecticut state statute, a specific approach to manage the skid-row alcoholic arriving repeatedly in the ER was developed. I report on this ER-based model and an approach to "capturing" the skid-row alcoholic.