denotes an abstract that is clinically relevant.
denotes that this is a recommended PHD Trainee Session.
denotes that this is a ticketed session.Hematology Disease Topics & Pathways:
Diseases, Immune Disorders, Treatment Considerations, Lymphoid Malignancies
Description:
Immune dysregulation often causes lymphadenopathy that requires clinicopathological correlation for accurate diagnosis. This session will review important causes of lymphadenopathy that fall outside the typical spectrum of malignant lymphoma. Castleman disease, IgG4-Related disease and the relationship between EBV and Post-transplant Lymphoproliferative Disorder will be reviewed. A common theme among these sessions is the importance of combining clinical, laboratory, and pathological “clues” to arrive at the best possible diagnosis and management plan.
Dr. Fajgenbaum will provide an overview on the classification, diagnosis, and management of Castleman disease. Castleman disease is a heterogeneous group of lymphoproliferative disorders, and idiopathic multicentric Castleman disease (iMCD) can be particularly difficult to diagnose and distinguish from other inflammatory causes of lymphadenopathy. Diagnostic criteria for iMCD and the subtypes of iMCD including thrombocytopenia, anasarca, (reticulin) fibrosis, renal dysfunction and organomegaly (iMCD-TAFRO), idiopathic plasmacytic lymphadenopathy (iMCD-IPL) and not otherwise specified (iMCD-NOS) will be reviewed.
Dr. Chen will review IgG4-related disease (IgG4-RD), a disease of immune dysregulation with protean manifestations including autoimmune pancreatitis, sclerosing cholangitis, lacrimal and salivary gland swelling, and tubulointerstitial nephritis. The session will focus on an approach to four key manifestations for Hematologists: Polyclonal hypergammaglobulinemia, IgG4-positive plasma cell enriched lymphadenopathy, eosinophilia, and retroperitoneal fibrosis. Practical tips for differentiating IgG4-RD from key mimickers such as Castleman disease, eosinophilic vasculitis, and histiocyte disorders will be highlighted.
Dr. Rouce will discuss the interplay between Epstein-Barr virus (EBV) and Post-tranplant lymphoproliferative disorder (PTLD). The spectrum of PTLD includes three variations of lymphoproliferation: lymphoid hyperplasia, neoplasia and malignancy. Lymphoid hyperplasia is characterized by polyclonal proliferation and non-destructive lesions whereas lymphoid malignancy features monoclonal proliferation and destructive lesions. EBV is the main driver of PTLD, and management ranges from reduction in immunosuppression to intensive, lymphoma-directed therapy.
Dr. Fajgenbaum will provide an overview on the classification, diagnosis, and management of Castleman disease. Castleman disease is a heterogeneous group of lymphoproliferative disorders, and idiopathic multicentric Castleman disease (iMCD) can be particularly difficult to diagnose and distinguish from other inflammatory causes of lymphadenopathy. Diagnostic criteria for iMCD and the subtypes of iMCD including thrombocytopenia, anasarca, (reticulin) fibrosis, renal dysfunction and organomegaly (iMCD-TAFRO), idiopathic plasmacytic lymphadenopathy (iMCD-IPL) and not otherwise specified (iMCD-NOS) will be reviewed.
Dr. Chen will review IgG4-related disease (IgG4-RD), a disease of immune dysregulation with protean manifestations including autoimmune pancreatitis, sclerosing cholangitis, lacrimal and salivary gland swelling, and tubulointerstitial nephritis. The session will focus on an approach to four key manifestations for Hematologists: Polyclonal hypergammaglobulinemia, IgG4-positive plasma cell enriched lymphadenopathy, eosinophilia, and retroperitoneal fibrosis. Practical tips for differentiating IgG4-RD from key mimickers such as Castleman disease, eosinophilic vasculitis, and histiocyte disorders will be highlighted.
Dr. Rouce will discuss the interplay between Epstein-Barr virus (EBV) and Post-tranplant lymphoproliferative disorder (PTLD). The spectrum of PTLD includes three variations of lymphoproliferation: lymphoid hyperplasia, neoplasia and malignancy. Lymphoid hyperplasia is characterized by polyclonal proliferation and non-destructive lesions whereas lymphoid malignancy features monoclonal proliferation and destructive lesions. EBV is the main driver of PTLD, and management ranges from reduction in immunosuppression to intensive, lymphoma-directed therapy.