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Biomedical subjects

C Costello

Publications and source records attributed to C Costello.

At least 73 records · Page 4Linked to original sources

Treatment of immune thrombocytopenic purpura in homosexual men.

Over the past 3 yr we have treated 6 homosexual men (age 22-55 yr) with immune thrombocytopenic purpura. 4 of the 6 have antibody to HTLV-III in their serum, 1 of these patients has the acquired immune deficiency syndrome (AIDS), 1 has AIDS-related-complex (ARC), and a 3rd has persistent generalised lymphadenopathy (PGL). The platelet count at presentation was between 2 and 35 X 10(9)/l and in each case a bone marrow confirmed active platelet production. Antiplatelet antibodies were demonstrated in 3 of 4 patients tested. 3 of the 6 patients showed a partial response to prednisolone, 2 showed little or no response and the 6th showed a good response. 2 patients received high dose i.v. immunoglobulin - 1 had an excellent response prior to splenectomy, the other showed no response. 5 of the 6 patients had a splenectomy. 3 had a lasting remission (12-27 months after splenectomy), 1 of these has HTLV-III antibodies; 1 had a remission lasting 1 yr, followed by fluctuating thrombocytopenia (21-130 X 10(9)/l) and 1 showed no response.

Adult↗

Pernicious anemia and systemic lupus erythematosus in a young woman.

We describe a 23-year-old woman with both pernicious anemia and systemic lupus erythematosus. This is a rare association despite the relationship between the autoimmune etiology of the 2 conditions, and it is of particular interest in that adult pernicious anemia is very uncommon in young people.

Adult↗

Midazolam versus hydroxyzine as intramuscular premedicant.

A randomized, double-blind, placebo-controlled, study was carried out in which the effects of midazolam (0.08 mg.kg-1) and hydroxyzine (1.5 mg.kg-1), with or without atropine (0.4 mg) or hyoscine (0.4 mg) were compared as intramuscular premedicants. Midazolam produced quicker onset of action, greater anxiolysis for the first hour, greater amnesia, less local irritation and a higher overall rating by the patients. Drowsiness, while also greater after midazolam, was neither marked nor prolonged. Both drugs were given similar overall ratings by the anaesthetists who administered the anaesthetics. Neither drug produced systemic toxicity. Of the two drugs known to produce amnesia, midazolam had a more profound effect and had an earlier onset than hyoscine. Midazolam (0.08 mg.kg-1) shows good potential as an intramuscular premedicant, especially when anaesthetic induction occurs 30 to 60 minutes later. Hyoscine, but not atropine, enhances the effects of the sedative drugs.

Adolescent↗

Cytoplasmic inclusions in a case of prolymphocytic leukemia.

A case of B-prolymphocytic leukemia (B-PLL) is reported in which intracytoplasmic inclusions were seen at transmission electron microscopy (TEM). The inclusions were electron dense, often membrane bound, and a proportion showed acid phosphatase reactivity.

Acid Phosphatase↗

Simultaneous or spontaneous occurrence of lympho- and myeloproliferative disorders: a report of four cases.

We describe four patients with mixed lympho- and myeloproliferative disorders. One patient had hairy cell leukaemia and acute myelomonocytic leukaemia, another lymphocytic lymphoma in leukaemic phase and chronic myelomonocytic leukaemia and the third patient had chronic lymphocytic leukaemia and polycythaemia rubra vera; none of these patients had received any prior therapy, and in two the diagnosis of the two malignancies was simultaneous. The fourth patient developed acute monocytic leukaemia 4 years after the diagnosis of chronic lymphocytic leukaemia after only 2 weeks of therapy with chlorambucil. The nuber of cases with concurrent or sequential but spontaneous occurrence of lympho- and myeloproliferative disorders reported so far is now 38. The questions relating to the pathogenesis of the two malignancies are discussed.

Aged↗

Hairy cell leukemia and myelomatosis: chance association or clinical manifestations of the same B-cell disease spectrum.

We describe three patients who had typical features of hairy cell leukemia (HCL) and multiple myeloma (MM) at the same time. In two, both diagnoses were made within a short period of time, and in the third, HCL had been present for 2 yr before the appearance of a paraprotein, bone lesions, and plasma-cell infiltrates established the diagnosis of MM. Although this association has not been previously reported, cases of HCL with osteolytic lesions or a paraprotein band have been described. The cases described may represent clinical manifestations of closely related disorders arising from divergent differentiation from a common B-cell precursor rather than a chance association.

Aged↗

Prolymphocytic leukaemia: an ultrastructural study of 22 cases.

Twenty-two cases of prolymphocytic leukaemia (PLL) have been studied by transmission electron microscopy (TEM); 17 had B-cell surface markers (B-PLL) and five had T-cell characteristics (T-PLL). The predominant cell, the prolymphocyte, has distinct features which were common to all cases: it is a relatively large lymphoid cell with a prominent nucleolus, well condensed peripheral nuclear chromatin and a variable amount of heterochromatin in intranuclear clumps. Eight cases of PLL (seven B-PLL, one T-PLL) showed minor morphological deviations from typical PLL which may present diagnostic difficulties when studied only at light microscopy: the cells in four of these had some features in common with those of chronic lymphocytic leukaemia (CLL), namely round regular nuclei and heavy chromatin condensation, but the nucleoli were larger in the PLL cases. In four other cases nuclear clefting was a prominent feature at TEM. B-PLL can be distinguished by ultrastructural features from other B-cell malignancies, e.g. follicular lymphoma, B-CLL and B-acute lymphoblastic leukaemia.

B-Lymphocytes↗

Ultrastructural cytochemistry of leukaemic cells: characterization of the early small granules of monoblasts.

An ultrastructural study of blast cells showing either monocytic or granulocytic differentiation was carried out with the acid phosphatase (AP) and myeloperoxidase (MPO) reactions. Eight cases of acute myeloid leukaemia (AML) and three of chronic granulocytic leukaemia in blast crisis were studied. A hitherto unrecognized small lysosomal granule characterized by AP activity and lack of MPO was present in the majority of cells of all six monoblastic leukaemias. These granules ranged from 0.05 to 0.2 micron in size and were distributed throughout the cytoplasm, frequently at the periphery of the cells. A small proportion of monoblasts showed AP reactivity in the Golgi cisternae. Both AP and MPO were positive in the granules of promonocytes; however, MPO positive granules were predominant in late promonocytes. Larger granules (0.2--0.6 micron) with MPO reactivity were characteristic of myeloblasts. In only two out of four cases did these granules show AP positivity, suggesting that, in contrast to monoblasts, AP activity is a late feature of myeloblastic differentiation. This study shows that ultrastructural cytochemistry may be helpful in the recognition and classification of acute leukaemias by demonstrating the early differentiation features of monocytic and granulocytic precursors.

Acid Phosphatase↗

Cell volume studies in B-cell leukaemia.

Leucocyte volume histograms were obtained in 12 cases of B-chronic lymphocytic leukaemia (B-CLL), two cases of B-prolymphocytic leukaemia (B-PLL) and two cases of hairy-cell leukaemia (HCL) by means of a Coulter model ZBl linked to a channelyser. Visual examination of the histograms showed differences between the cases which were confirmed by measurement of the modal volume (MV) and the sigma value (SD of log-volume). The MV of the cells in B-CLL ranged from 153.8 to 244.2 fl (mean 198.24 fl) and was lower than the MV in the cases of HCL (427.5 and 465.6 fl). One case of B-PLL had cells with an MV within the B-CLL range while in the other the MV was greater (317.9 fl). The histogram in one of two cases of B-CLL in 'prolymphocytoid' transformation showed a relatively high sigma value, thus suggesting heterogeneity in cell size. The volume histogram is a simple method of sizing the predominant cell in the peripheral blood in lympho-proliferative disorders and permits an objective comparison between cell sizes in different diseases.

B-Lymphocytes↗

Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis.

A boy with glutaric acidemia had psychomotor retardation first noted at age 6 months, recurrent metabolic acidosis, and a progressive quadriparesis with choreoathetosis. He died at age 3 1/2 years. Cultured skin fibroblasts lacked glutaryl-CoA dehydrogenase activity. There was a biochemical, but not a clinical, response to dietary restriction of lysine and tryptophan. The caudate and putamen of the brain showed severe loss of nerve cells and fibers with proliferation of astrocytes, as well as markedly reduced gamma-aminobutyric acid and glutamate decarboxylase activity.

Acidosis↗

Cytochemistry of normal and leukaemic lymphocytes: a review.

Findings with six cytochemical reactions demonstrable in normal and leukaemic lymphocytes were reviewed. The two methods which are presently of greater diagnostic value are the acid phosphatase (AP) and alpha-naphthyl acetate esterase (ANAE) reactions. AP has a definitive role in the diagnosis of acute and chronic T-cell leukaemias, where a strong positive reaction helps to distinguish them from most B-cell lymphoproliferative disorders. New findings concerning the ultrastructural localization of this enzyme are presented. ANAE is of value in distinguishing T-lymphocytes (positive localized reaction) from B lymphocytes (negative reaction) and the T micron from the T gamma subpopulation of T-lymphocytes, a positive reaction demonstrable only in the T micron cells. Other reactions reviewed were PAS, beta-glucoronidase, hexosaminidase and alkaline phosphatase.

Acid Phosphatase↗

Analysis and quantitation of free ceramide containing nonhydroxy and 2-hydroxy fatty acids, and phytosphingosine by high-performance liquid chromatography.

Reaction of ceramides containing nonhydroxy fatty acids with benzoyl chloride in pyridine at 70 degrees C for 1 hr resulted in N-benzoylation to form N,N-acyl,benzoyl derivatives; O-benzoylation also occurred. However with ceramides containing 2-hydroxy fatty acids and phytosphingosine only O-benzoylation occurred even on prolonged treatment. Only O-benzoylation occurred on reaction with benzoic an hydride. However, the benzoylation of ceramides with phytosphingosine could not be achieved with benzoic anhydride and this benzoylation was performed by reaction with benzoyl chloride at 70 degrees C for 4 hr. Because N,N-acyl,benzoyl derivatives of ceramides containing nonhydroxy fatty acids produced by treatment with benzoyl chloride overlap methyl benzoate on high-performance liquid chromatography, benzoic anhydride was preferable for benzoylation of ceramides with nonhydroxy and 2-hydroxy fatty acids. On the other hand, the reaction with benzoyl chloride at 70 degrees C for 4 hr was used for quantitation of benzoylated ceramides containing 2-hydroxy fatty acids and phytosphingosine. 3-(p-Phenylbenzoyl)estrone was used as an internal standard for both reactions and values for ceramides containing 2-hydroxy fatty acids obtained by the two reactions were in good agreement. This procedure was applied to measurement of the ceramide levels in the brain, liver, and kidney of rats during development. The levels of ceramides containing nonhydroxy and 2-hydroxy fatty acids in the brain, liver, and kidney increased to the adult levels and then remained unchanged. Ceramide with phytosphingosine was detected in the liver and kidney, where its concentration gradually increased with age, but it was not found in the brain. The composition of nonhydroxy fatty acids were also analyzed.

Aging↗