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Biomedical subjects

M Bernadette Garvey

Publications and source records attributed to M Bernadette Garvey.

7 recordsLinked to original sources

A patient with anemia of obscure origin: Crohn's disease in disguise.

BACKGROUND: A 65-year-old white Mediterranean male with a 10-year history of intermittent anemia, who was otherwise completely asymptomatic, was referred to our hospital in March 2004. He had a medical history of beta thalassemia and fecal occult blood tests had occasionally been positive. INVESTIGATIONS: Fecal occult blood test, laboratory investigations, esophagogastroduodenoscopy, colonoscopy with retrograde ileoscopy, mesenteric angiography, small-bowel series, CT scan of the abdomen and pelvis, Meckel's scan, and capsule endoscopy. Laparoscopic surgery followed by macroscopic and microscopic histopathologic examination of samples obtained during the procedure. DIAGNOSIS: Crohn's disease of the small bowel. MANAGEMENT: Laparoscopic segmental small-bowel resection with end-to-end anastomosis. Postsurgical treatment with Pentasa 4 g a day.

Aged↗

Gamma testing experience with the ACL TOP hemostasis testing system.

As a gamma testing site or a limited early release site for the ACL TOP, St. Michael's Hematology Laboratory evaluated the ACL TOP for its ability to fit into a laboratory whose workflow includes large volumes of routine and specialty hemostasis assays. This evaluation included the determination of the ACL TOP's precision, normal ranges, and reagent sensitivities. Analytical correlation studies for the ACL TOP were performed in comparison to the ACL ADVANCE. The ACL TOP was also tested for its ability to handle large volumes of not only routine assays but also more specialized coagulation assays. Instrument precision, normal reference range assignment, and factor sensitivities met the requirements of this laboratory. The ACL TOP correlated favorably to the ACL ADVANCE, and in a workup for thrombophilia its throughput was twice what was seen with the ACL ADVANCE. The speed of the ACL TOP was impressive, generating results at a rate of almost 5 results/min. In this gamma testing study, the ACL TOP has demonstrated suitability as a precise coagulation analyzer for use in the settings of a high-volume, fast-paced, specialized coagulation laboratory faced with demanding turnaround times.

Autoanalysis↗

Immunoadsorption of factor VIII inhibitors.

PURPOSE OF REVIEW: Extracorporeal immunoadsorption is being increasingly applied in a variety of disorders. This approach is particularly suited to removal of antibodies or inhibitors to coagulation factor VIII and may be particularly useful before the administration of large amounts of expensive replacement or bypass therapy for patients with hemophilia who are bleeding, or patients undergoing immune tolerance therapy. RECENT FINDINGS: In patients with inhibitors to factor VIII, several types of immunoadsorption therapy have been used, although reports are mainly anecdotal, consisting of relatively small numbers of persons. Nonetheless, the findings suggest that immunoadsorption may be clinically effective and cost-effective and should be considered early in the treatment of appropriate patients. New immunoadsorption technologies are being described for a variety of disorders, including hemophilia, and a new synthetic matrix of polystyrene beads functionalized with sulfonate and tyrosyl methylester groups for immunoadsorption removal of factor VIII inhibitors is intriguing. SUMMARY: Although immunoadsorption was shown to be clinically effective in patients with inhibitors to factor VIII more than two decades ago, recent papers have emphasized the desirability of early implementation of the modality in the treatment plan. Immunoadsorption is relatively easy to perform with few adverse effects, but specialized equipment is required, and it should be performed in an experienced setting. Although potentially less costly than other (bypass) therapies, immunoadsorption is itself not inexpensive, and its comparative effectiveness with plasmapheresis and other management options for the dangerously bleeding patient with antibodies to factor VIII should be determined by multicenter randomized controlled trials. Interesting recent novel technical developments in the field may facilitate increased use of the procedure.

Factor VIII↗

Thrombotic thrombocytopenic purpura associated with a prolactin-producing pituitary adenoma.

We report here the case of a 44-year-old woman with thrombocytopenia, anemia, convulsions, hyperprolactinemia, and galactorrhea. The patient died of cardiac failure. Autopsy revealed PAS-positive and von Willebrand factor-positive microthrombi in the arterioles and capillaries of many organs, mainly in the heart and brain, confirming the clinical diagnosis of thrombotic thrombocytopenic purpura. In the pituitary, a prolactin-producing adenoma was identified. To our knowledge, thrombotic thrombocytopenic purpura accompanied by a prolactin-secreting pituitary adenoma has not yet been described. The question of whether the association between the vascular changes and the pituitary adenoma is incidental or causal cannot be answered. Further studies are required to determine whether prolactin released from the pituitary tumor in excess played a role in the formation of microthrombi causing multiple organ failure and the demise of the patient.

Adult↗

Immunoadsorption may provide a cost-effective approach to management of patients with inhibitors to FVIII.

BACKGROUND: Immunoadsorption of plasma with Staphylococcal protein A removes immunoglobulins and immune complexes; hence, it should effectively remove inhibitors to FVIII in acquired or congenital hemophilia. The procedure may be cost effective, given the expense of therapies used to treat patients with inhibitors, particularly in an acute setting. STUDY DESIGN AND METHODS: Three patients with inhibitors to FVIII were treated with the Excorim Immunosorba system (two columns used in tandem). Costs for immunoadsorption and for other therapeutic products administered to the patients were calculated. RESULTS: Two patients had acquired hemophilia and severe bleeding associated with low levels of circulating FVIII and high levels of inhibitors to FVIII. They failed to achieve a satisfactory response to management with immunosuppression, pFVIII, recombinant FVIIa or IVIG but responded rapidly, with long-term benefit, to immunoadsorption therapy. The third patient had congenital hemophilia and immunoadsorption was effective in reducing his inhibitor level, allowing him to undergo immune tolerance therapy. Costs of treatment before immunoadsorption were markedly higher than those associated with the immunoadsorption procedures (i.e., >Can 350,000 dollars and >Can 1,000,000 dollars vs. < 20,000 dollars). CONCLUSION: Immunoadsorption appears to be an effective and cost-effective alternative in the management of patients with inhibitors to FVIII.

Adolescent↗

Cellular immune mechanisms in autoimmune thrombocytopenic purpura: An update.

Autoimmune thrombocytopenic purpura (AITP) is a bleeding disorder in which autoantibodies are directed against an individual's own platelets, leading to enhanced clearance through Fc receptor (R)-mediated phagocytosis by macrophages residing in the reticuloendothelial system (RES), particularly in the spleen. The production of IgG autoantibodies is critically dependent on cellular immune mechanisms particularly relating to T cells. We review the recent literature of the cell-mediated immunology of AITP focusing on platelet phenotype, genetics, T-cell reactivities, and cytokine profiles in patients with AITP. Understanding the interaction between these cell-mediated mechanisms is vital for developing antigen specific immunotherapies to treat this autoimmune disease.

Autoantibodies↗