From the Department of Medicine, Division of Translational Medicine and Human Genetics, Center for Cytokine Storm Treatment and Laboratory (D.C.F.), and the Center for Cellular Immunotherapies and the Parker Institute for Cancer Immunotherapy (C.H.J.), Perelman School of Medicine, University of Pennsylvania, Philadelphia. Address reprint requests to Dr. Fajgenbaum at davidfa@ pennmedicine . upenn . edu or to Dr. June at cjune@ upenn . edu.

Cytokine Storm.

A panel of experts was convened by the Infectious Diseases Society of America (IDSA) and Society for Healthcare Epidemiology of America (SHEA) to update the 2010 clinical practice guideline on Clostridium difficile infection (CDI) in adults. The update, which has incorporated recommendations for children (following the adult recommendations for epidemiology, diagnosis, and treatment), includes significant changes in the management of this infection and reflects the evolving controversy over best methods for diagnosis. Clostridium difficile remains the most important cause of healthcare-associated diarrhea and has become the most commonly identified cause of healthcare-associated infection in adults in the United States. Moreover, C. difficile has established itself as an important community pathogen. Although the prevalence of the epidemic and virulent ribotype 027 strain has declined markedly along with overall CDI rates in parts of Europe, it remains one of the most commonly identified strains in the United States where it causes a sizable minority of CDIs, especially healthcare-associated CDIs. This guideline updates recommendations regarding epidemiology, diagnosis, treatment, infection prevention, and environmental management.

/pdf/clinical-practice-guidelines-for-clostridium-difficile-4hs0j01jnf.pdf

Clinical Practice Guidelines for Clostridium difficile Infection in Adults and Children: 2017 Update by the Infectious Diseases Society of America (IDSA) and Society for Healthcare Epidemiology of America (SHEA)

We herein present an overview of the upcoming 5th edition of the World Health Organization Classification of Haematolymphoid Tumours focussing on lymphoid neoplasms. Myeloid and histiocytic neoplasms will be presented in a separate accompanying article. Besides listing the entities of the classification, we highlight and explain changes from the revised 4th edition. These include reorganization of entities by a hierarchical system as is adopted throughout the 5th edition of the WHO classification of tumours of all organ systems, modification of nomenclature for some entities, revision of diagnostic criteria or subtypes, deletion of certain entities, and introduction of new entities, as well as inclusion of tumour-like lesions, mesenchymal lesions specific to lymph node and spleen, and germline predisposition syndromes associated with the lymphoid neoplasms. 

/pdf/the-5th-edition-of-the-world-health-organization-1mm2sff3.pdf

The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

A defining property of cytotoxic lymphocytes is their expression and regulated secretion of potent toxins, including the pore-forming protein perforin and serine protease granzymes. Until recently, mechanisms of pore formation and granzyme transfer into the target cell were poorly understood, but advances in structural and cellular biology have now begun to unravel how synergy between perforin and granzymes brings about target cell death. These and other advances are demonstrating the surprisingly broad pathophysiological roles of the perforin–granzyme pathway, and this has important implications for understanding immune homeostasis and for developing immunotherapies for cancer and other diseases. In particular, we are beginning to define and understand a range of human diseases that are associated with a failure to deliver active perforin to target cells. In this Review, we discuss the current understanding of the structural, cellular and clinical aspects of perforin and granzyme biology.

Perforin and granzymes: function, dysfunction and human pathology

We report the updated classification of Inborn Errors of Immunity/Primary Immunodeficiencies, compiled by the International Union of Immunological Societies Expert Committee. This report documents the key clinical and laboratory features of 430 inborn errors of immunity, including 64 gene defects that have either been discovered in the past 2 years since the previous update (published January 2018) or were characterized earlier but have since been confirmed or expanded upon in subsequent studies. The application of next-generation sequencing continues to expedite the rapid identification of novel gene defects, rare or common; broaden the immunological and clinical phenotypes of conditions arising from known gene defects and even known variants; and implement gene-specific therapies. These advances are contributing to greater understanding of the molecular, cellular, and immunological mechanisms of disease, thereby enhancing immunological knowledge while improving the management of patients and their families. This report serves as a valuable resource for the molecular diagnosis of individuals with heritable immunological disorders and also for the scientific dissection of cellular and molecular mechanisms underlying inborn errors of immunity and related human diseases.

/pdf/human-inborn-errors-of-immunity-2019-update-on-the-38qelf7u12.pdf

Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee

Biologics can be defined as any agent derived from a living organism or natural substance. Vaccines, blood components, allergens, somatic cells, gene therapy, tissues, and recombinant therapeutic proteins are all included in this category. This chapter will focus on cell-based therapy other than hematopoietic stem cell transplant and modifiers of immune responses.

Biologics and therapeutics

As overall outcomes from hematopoietic stem cell transplantation (HSCT) continue to improve, the repertoire of nonmalignant indications for transplant also continues to expand. Nonmalignant conditions accounted for approximately 30% of all transplants performed in the USA from 2009–2013 with this number expected to continue rising. In addition, HSCT is now being performed for many disorders for which it was previously reserved for severe clinical phenotypes only. This chapter provides an overview of the transplant process for nonmalignant disorders (NMD). It highlights the unique experiences of NMD HSCT based on disease category: primary immunodeficiency syndromes, hemoglobinopathies and disorders of red cells, inherited disorders of metabolism, and aplastic anemia and bone marrow failure syndromes. Furthermore, it touches on recent developments in reduced intensity conditioning regimens, GVHD prophylaxis, and the management of post-transplant infection risks.

Overview of Hematopoietic Stem Cell Transplantation for Nonmalignant Diseases

Third-Party Virus-Specific T-Cell Infusion for the Treatment of Refractory Viral Infections: Results from PBMTC SUP1701

Hematopoietic Cell Transplant for Patients With NEMO Defect: A Single Center Experience

The use of elevated ferritin levels in neonates and young infants as a screening lab for HLH

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