The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc.

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue

From N Engl J Med, Hideaki Tsukuma, Akihisa Imagawa, M.D., Toshiaki Hanafusa, M.D., Ph.D., Jun-ichiro Miyagawa, M.D., Ph.D., and Yuji Matsuzawa, M.D., Ph.D., A Novel Subtype of Type 1 Diabetes Mellitus Characterized by a Rapid Onset and an Absence of Diabetes-Related Antibodies, 342:301-307. Copyright © 2000 Massachusetts Medical Society. Reprinted with permission.

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A novel subtype of type 1 diabetes mellitus characterized by a rapid onset and an absence of diabetes-related antibodies

Background— Little is known about the relationship between intrinsic cardiac nerve activity (ICNA) and spontaneous arrhythmias in ambulatory animals. Methods and Results— We implanted radiotransmitters to record extrinsic cardiac nerve activity (ECNA; including stellate ganglion nerve activity and vagal nerve activity) and ICNA (including superior left ganglionated plexi nerve activity and ligament of Marshall nerve activity) in 6 ambulatory dogs. Intermittent rapid left atrial pacing was performed to induce paroxysmal atrial fibrillation or atrial tachycardia. The vast majority (94%) of ligament of Marshall nerve activity were preceded by or coactivated with ECNA (stellate ganglion nerve activity or vagal nerve activity), whereas 6% of episodes were activated alone without concomitant stellate ganglion nerve activity or vagal nerve activity. Paroxysmal atrial fibrillation and atrial tachycardia were invariably (100%) preceded (<5 seconds) by ICNA. Most paroxysmal atrial tachycardia events (89%) were prec...

Intrinsic Cardiac Nerve Activity and Paroxysmal Atrial Tachyarrhythmia in Ambulatory Dogs

AIMS We examined the left lateral ridge (LLR) between the orifices of the left pulmonary veins and the left atrial appendage for a better understanding of its structural composition relevant to ablations for atrial fibrillation (AF). METHODS AND RESULTS The LLR and its surrounding areas were studied in 40 heart specimens by dissection and histological sections. The LLR is a fold of the atrial wall with a mean width that was narrower superiorly than inferiorly (P < 0.001). Its myocardial thickness at the antero-superior level was thicker than at the postero-inferior level (2.8 +/- 1.1 vs. 1.7 +/- 0.8 mm, P < 0.001). Transmurally from subepicardium to subendocardium, the LLR comprises myofibres from the leftward extension of Bachmann's bundle together with the inferior branches of the septopulmonary bundle and the septoatrial bundle. The vein or ligament of Marshall is located on the epicardial aspect of the LLR. The Marshall structures and autonomic nervous system are in close proximity (<3 mm) to the endocardial surface at the superior level of the ridge in 70% of specimens. CONCLUSION The variability in width and thickness of the LLR, its proximity to Marshall structures and autonomic nerves, and myofibre arrangement may be significant in the fibrillatory process and spread of AF activity.

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The architecture of the left lateral atrial wall: A particular anatomic region with implications for ablation of atrial fibrillation

Atrial fibrillation (AF) causes important mortality and morbidity on a population-level. So far, we do not have the means to prevent AF or AF-related complications adequately. Therefore, over 70 experts on atrial fibrillation convened for the 2nd AFNET/EHRA consensus conference to suggest directions for research to improve management of AF patients (Appendix 1). The group defined three main areas in need for research in AF: 1. better understanding of the mechanisms of AF; 2. Improving rhythm control monitoring and management; and 3. comprehensive cardiovascular risk management in AF patients. The group put forward the hypothesis that successful therapy of AF and its associated complications will require comprehensive therapy. This applies e.g. to the "old" debate of "rate versus rhythm control", since rhythm control is generally added to underlying (continued) rate control therapy, but also to the emerging debate of "antiarrhythmic drugs versus catheter ablation", of which both may be needed in most patients to maintain sinus rhythm, but also to therapy of conditions that predispose to AF and contribute to cardiovascular complications such as stroke, cognitive decline, heart failure, and acute coronary syndromes. We call for research initiatives aiming at a better understanding of the different causes of AF and its complications, and at development and validation of mechanism-based therapies. The future of AF therapy may require a combination of management of underlying and concomitant conditions, early and comprehensive rhythm control therapy, adequate control of ventricular rate and cardiac function, and continuous therapy to prevent AF-associated complications (e.g. antithrombotic therapy). The reasons for these suggestions are detailed in this paper.

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Early and comprehensive management of atrial fibrillation: executive summary of the proceedings from the 2nd AFNET-EHRA consensus conference 'research perspectives in AF'.

Ligament of Marshall. Introduction: The ligament of Marshall (LOM) or the oblique vein of Marshall (VOM) in the left atrium (LA) is one of the origins of nonpulmonary vein ectopies causing atrial fibrillation. The distributions of myocardial bundles (Marshall bundles; MBs) and autonomic nerves adjacent to the LOM have not been completely elucidated. Methods and Results: Twenty-eight human hearts were examined postmortem. The inferolateral LA myocardium was dissected en block and cut perpendicularly to the atrioventricular ring. The specimens were sectioned at 1-mm intervals and stained with hematoxylin and eosin, and Azan-Mallory. In addition, S-100 and tyrosine hydroxylase (TH) were employed for the immunohistochemical analysis of the sympathetic and parasympathetic fibers. In 25 cases, MBs were observed. Although the MBs surrounded the VOM at the coronary sinus (CS) juncture, they gradually diminished in density toward the distal venous branch. The connections of the MBs and LA myocardium were frequently observed in the CS juncture and anterior wall of the left pulmonary vein (PV)-LA junctions. TH-stained sympathetic nerve fibers were densely distributed around the PV-LA junctions. On the other hand, non-TH-stained parasympathetic ganglions were mainly observed at the CS juncture. Conclusions: MB-LA connections were mainly observed at the CS juncture and PV-LA junctions. The association between sympathetic nerve fibers and MBs was distinct at the PV-LA junctions. At the CS juncture, the regression of the sympathetic nerve fibers and the increase of parasympathetic ganglions were observed. The diverse arrangement and autonomic innervation of MBs seem to characterize the LOM region in humans.

Diverse myocardial extension and autonomic innervation on ligament of Marshall in humans.

Lanthanum carbonate is one of the new phosphate binders used for the treatment of hyperphosphatemia in patients with chronic kidney disease. It is poorly absorbed from the gastrointestinal tract, forms insoluble complexes within the lumen, and prevents the absorption of dietary phosphate. A 63-year-old female with a 7-year history of peritoneal dialysis, who was treated with lanthanum carbonate for four years, underwent endoscopic submucosal dissection for intramucosal gastric cancer. Resected specimens showed massive accumulation of macrophages containing fine, granular, brown material in the lamina propria. This was confirmed as lanthanum deposition by scanning electron microscopy with energy dispersive x-ray spectroscopy. Although lanthanum may be poorly absorbed, increased tissue accumulation of lanthanum, particularly in the liver and bone, has been reported in animals with chronic kidney disease. This report indicates enhanced gastrointestinal absorption of lanthanum in some patients or conditions, although its clinical significance awaits further studies.

Extensive lanthanum deposition in the gastric mucosa: the first histopathological report.

We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were detected. Genetic analysis revealed that mutation of p.Arg1215Gln in CFH was identified. The diagnosis of aHUS was confirmed and eculizmab therapy was currently effective for 5 months.

Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation and IgA Nephropathy: A Case Report Successfully Treated with Eculizumab.

To assist physicians, especially young physicians, in identifying tuberculosis (TB) infection before the terminal stage, we analyzed 7 cases of numerous tuberculous granulomas in multiple organs and compared clinical and autopsy findings between cases. Patients ranged in age from 41 to 86 years at the time of death. The main chief complaint was fever of unknown origin (3 of 7 cases [43%]). The main underlying conditions were liver cirrhosis (2 of 7 cases [29%]) and chronic renal failure (2 of 7 cases [29%]). Two patients (29%) had been given methylprednisolone pulse therapy for various lung disorders. Active TB was not diagnosed before autopsy in 4 of 7 (57%) patients. Calcified lesions indicative of old TB were present in 4 of 7 (57%) patients. Thus, miliary tuberculosis may represent a re-emergence of latent TB infection in these cases. Various histologic features of nonreactive exudative inflammation were seen, along with granulomas containing Langhans giant cells with or without caseous necrosis in hypervascular organs, such as the lung, liver, and bone marrow. Physicians should be mindful of the possibility of miliary TB when older patients with hepatorenal disease and a history of TB infection have undergone immunosuppressive treatment. Active tuberculous infection can depend on the presence of an underlying disease and immunocompromise.

Autopsy cases of miliary tuberculosis: clinicopathologic features including background factors.

A previously healthy middle-aged woman noted a rapid onset of flank pain with gross hematuria. Enhanced CT scan showed thrombosis of the inferior vena cava and right renal vein. Laboratory findings revealed nephrotic proteinuria, Sjogren's syndrome (SjS), and Graves' disease (GD). A right nephrectomy was performed because of progressive and refractory renal necrosis. Renal specimens showed venous infarction with diffuse hemorrhagic and severe congestive renal necrosis, and membranous nephropathy (MN). The present case was diagnosed as acute renal necrosis due to catastrophic thrombosis in a patient with SjS, GD, and MN. It was thought that sudden development of thrombosis may have been caused by the status of the autoimmune disorders, and the associated MN.

Mutsuki Makino

Papers

Diverse myocardial extension and autonomic innervation on ligament of Marshall in humans.

Extensive lanthanum deposition in the gastric mucosa: the first histopathological report.

Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation and IgA Nephropathy: A Case Report Successfully Treated with Eculizumab.

Autopsy cases of miliary tuberculosis: clinicopathologic features including background factors.

A Fulminant Case of Renal Vein Thrombosis in a Patient with Autoimmune Disorder and Membranous Nephropathy