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Biomedical subjects

R Manley

Publications and source records attributed to R Manley.

17 recordsLinked to original sources

Clonally unrelated BCR-ABL-negative acute myeloblastic leukemia masquerading as blast crisis after busulphan and interferon therapy for BCR-ABL-positive chronic myeloid leukemia.

We report a patient with Philadelphia (Ph)-positive, BCR-ABL rearrangement positive, chronic myeloid leukemia (CML) with a prolonged chronic phase of 24 years who was first prescribed alpha-2 interferon 22 years after initial diagnosis. This therapy was tolerated poorly on account of thrombocytopenia, but an eventual major cytogenetic response was followed soon afterwards by transformation to terminal acute myeloid leukemia (AML). Cytogenetic studies indicated that the transformed myeloblasts were karyotypically normal and Ph negative. Although polymerase chain reaction (PCR) analysis of total leukemic mRNA remained BCR-ABL positive, other molecular studies, including Southern blotting and fluorescent in situ hybridization (FISH) analyses, showed that myeloblasts were BCR-ABL rearrangement negative. PCR-based clonality studies using an X-chromosome-linked restriction fragment polymorphism within the phosphoglycerate kinase gene (PGK1) further showed that the Ph-negative blast cells had a different clonal origin from the Ph-positive clone of chronic phase. We suggest that cases of underlying Ph-negative leukemic transformation in Ph-positive CML warrant further study and should be considered for trial of intensive remission induction therapy as appropriate for acute leukemia.

Acute Disease

Co-incidental presentation of IgA lambda multiple myeloma and pleural involvement with IgM kappa non-Hodgkin's lymphoma.

Pleural effusions occur in approximately 6% of patients with myeloma. The aetiology is multifactorial and effusions due to pleural myelomatous involvement are rare, occurring in < 1% of cases. We report the case of a 68-year-old lady who presented with IgA myeloma and a concurrent pleural effusion due to a second IgM kappa producing B cell neoplasm. The former responded but the latter was resistant to standard myeloma therapy.

Aged

Polyploidy in myelodysplastic syndrome: a case report.

Structural chromosomal abnormalities are common in myelodysplastic syndromes (MDSs), and complex abnormalities are known to confer a poor prognosis. Polyploidy is rare. We report a patient with MDS in whom 8/15 cells were 77,XYY; 2/15 were 83,XYY, and 5/15 were diploid (46,XY). He experienced rapid evolution of disease, transforming to terminal acute leukemia.

Acute Disease

An assessment of the effectiveness of the revised FDA checklist.

In 1978, the Food and Drug Administration (FDA), developed a generic anesthesia equipment preuse checklist. The checklist was first released by the FDA in August 1986 and endorsed by the American Association of Nurse Anesthetists on October 18, 1986. The FDA checklist was revised in 1992 to improve the abilities of anesthesia providers to detect machine faults. In the present study, the investigators attempted to determine the effectiveness of the revised FDA checklist in detection of anesthesia machine faults as compared to providers' usual methods. Whereas no published study of preanesthesia safety inspection had been performed since the revision of the FDA checklist, the authors compared the detection abilities of anesthesia providers before and after inclusion of the revised FDA checklist. Twenty-two anesthesia providers were tested to compare the number of prearranged anesthesia machine faults that could be detected with (1) their usual checkout methods, and (2) with the revised FDA checklist. Data describing the subjects' fault detection abilities were analyzed using the t test for paired observation (P value < 0.05 considered significant). Statistical analysis revealed no significant difference (P = 0.479) when subjects used the FDA checklist and when they used their usual method. Use of the FDA machine checklist was no more effective than the provider's usual method in discovering machine faults. When using their normal method, 54.5% of providers did not discover more than 50% of programmed faults. Approximately 40.9% of providers who used the revised FDA checklist did not discover over 50% of programmed faults.

Anesthesiology