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Background Controlled trials have found therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIg) infusion therapy to be equally efficacious in treating Guillain-Barré syndrome (GBS). Due to increases in the price of IVIg compared to human serum albumin (HSA), used as a replacement fluid in TPE, we examined direct hospital-level expenditures for TPE and IVIg for meaningful cost-differences between these treatments. Methods Using financial data from our two institutions, hospital cost profiles for IVIg and 5% albumin were established. Reimbursement amounts were obtained from publicly available Medicare data resources to determine payment rates for TPE, non-tunneled central catheter line placement, and drug infusion therapy. A model was developed which allows hospitals to input cost and reimbursement amounts for both IVIg and TPE with HSA that results in real-time valuations of these interventions. Results The direct cost of five IVIg infusion sessions totaling 2.0 grams per kilogram (g/kg) body weight was $10,329.85 compared to a series of five TPE procedures, which had direct costs of $4,638.16. Conclusions In GBS patients, direct costs of IVIg therapy are more than twice that of TPE. Given equivalent efficacy and similar severity and frequencies of adverse events, TPE appears to be a less expensive first-line therapy option for treatment of patients with GBS.

Thrombotic thrombocytopenic purpura (TTP) rarely occurs with systemic vasculitis. A 17-year-old girl presented with non-bloody diarrhea, menorrhagia, and syncope. She had severe anemia (hemoglobin = 3.8 g/dl), thrombocytopenia (platelet = 7,000/mm 3 ), and acute kidney injury (serum creatinine, Cr = 2.3 mg%). Peripheral smear examination confirmed the presence of microangiopathic hemolytic anemia. Additionally, she had a positive anti-nuclear antibody (1:1600) and normal complement levels. We considered the diagnosis of TTP, possibly associated with systemic lupus erythematosus, and promptly initiated pulse methylprednisolone and daily 3–4 l of plasma exchange therapy. Following resolution of her thrombocytopenia in 48 h, we performed a kidney biopsy that revealed diffuse proliferative, focal crescentic, and necrotizing glomerulonephritis with mild IgG immunofluorescence staining. Concomitantly, autoimmune work-up was significant for positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA = 1:640) and decreased von Willebrand factor cleaving protease activity (<5%). A final diagnosis of TTP with microscopic polyangiitis (p-ANCA-mediated) was made and treatment with daily oral cyclophosphamide and prednisone resolved her renal injury over 2 months (follow-up Cr = 1.0 mg%). Our case highlights the importance of identifying systemic disorders such as ANCA-associated vasculitis with TTP.

In patients with immune thrombotic thrombocytopenic purpura (iTTP), autoantibodies against the metalloprotease ADAMTS13 lead to catastrophic microvascular thrombosis. However, the potential benefits of recombinant human ADAMTS13 (rADAMTS13) in patients with iTTP remain unknown. Here, we report the clinical use of rADAMTS13, which resulted in the rapid suppression of disease activity and complete recovery in a critically ill patient whose condition had proved to be refractory to all available treatments. We also show that rADAMTS13 causes immune complex formation, which saturates the autoantibody and may promote its clearance. Our data support the role of rADAMTS13 as a novel adjunctive therapy in patients with iTTP. In a 28-year-old woman with postpartum immune thrombotic thrombocytopenic purpura, plasma exchange was unsuccessful. Near death while receiving vasopressors, she was treated with recombinant ADAMTS13, and the disease abated.

Throughout the history of hemotherapy (HT), various challenges and concerns have been encountered in its practical application. When viewed using a prismatic lens of history, recurrent themes regarding adverse HT sequelae separate and become apparent. These can be broadly classified into three categories: infectious, noninfectious, and administrative/logistical. Using the HT care map as a frame of reference along with its associated rites, we examine the contemporary spectrum of HT adverse events and concerns, and some approaches as to how these may be addressed from bedside and laboratory medicine biovigilance perspectives enhancing patient care and blood transfusion safety. Although our vantage point is from an academic community hospital venue, the issues and concerns identified are germane to many if not all transfusion-medicine practice environments. Included among the subjects we explore are patient/specimen identification issues, blood-management initiatives, unrecognized and/or unreported suspected transfusion reactions, transfusion-associated adverse pulmonary sequelae (including transfusion-related acute lung injury and transfusion-associated circulatory overload), expanded applications of electronic health records and issues regarding their “meaningful use” and interinstitutional “digital compatibilities”, biovigilance integration of electronic data networks within and between health care entities, and anticipated workforce contractions secondary to projected declines in the availability of qualified laboratory professionals. Cooperative initiatives between accreditation and regulatory entities, blood collectors and suppliers, hospital laboratory transfusion services and health care providers involved in the bedside care of the patient offer the best means in further mitigating adverse HT sequelae and potential nonconformities that may arise in the daily operations of contemporary hospital blood banks and transfusion-medicine services.

There has been a worldwide increase in the incidence of asthma, and the disease has greatly impacted the public health care system. Chlamydia pneumoniae has been reported as a possible contributing factor in asthma. The organism has been detected by polymerase chain reaction (PCR) in bronchial tissue, but there has been no direct evidence of viability. To determine the frequency of viable Chlamydia in children, blood and bronchoalveolar lavage were collected from 70 pediatric patients undergoing flexible fiberoptic bronchoscopy. Forty-two of these patients had asthma, whereas the remaining patients had various respiratory disorders. Fifty-four percent (38) of the bronchoalveolar lavage samples were PCR-positive for Chlamydia, and 31% (22) of the PCR-positive samples were positive when cultured on macrophages. Twenty-eight samples (40%) and 14 samples (20%) of the PCR- and culture-positive samples, respectively, were from patients with asthma. Culture of the blood samples revealed that 24 (34.3%) of 70 were positive for Chlamydia compared with 8 (11%) of 70 matched nonrespiratory control subjects (p < 0.01); 17 (24%) of the positive blood cultures from the respiratory group were from patients with asthma. Elevation of total IgE was strongly associated with lavage culture positivity for Chlamydia. We therefore conclude that viable Chlamydia pneumoniae organisms are frequently present in the lung lavage fluid from this cohort of predominantly asthmatic pediatric patients.