To most, the complement system resides in one of the dark corners of hematology, dimly illuminated occasionally when the direct antibody test is reported as “positive” for C3 in a patient with autoimmune hemolytic anemia. Undeniably, the complement system, with its three overlapping pathways (classical, alternative, and lectin), is complex. But the development of new and emerging targeted therapies necessitates understanding of this arcane system so as to allow development of an appropriate management plan for patients with complement-mediated hematologic disorders.
Dr. Charles Parker will provide an update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria (PNH). Once suspected, the diagnosis of PNH is straightforward, but PNH is not a binary disease, and management remains challenging because of the heterogeneous nature of the disease and its close association with bone marrow failure. The lecture will focus on an approach to subcategorizing PNH that can serve as a framework for management of this complex disease.
Dr. Vahid Afshar-Kharghan will summarize the advances in understanding the pathogenesis and natural history of atypical hemolytic uremic syndrome (aHUS) in light of available information about the genetics of the disorder. He will discuss diagnostic uncertainties in differentiating aHUS from other thrombotic microangiopathies and will present a practical framework for diagnosis and management of aHUS.
Dr. Sigbjørn Berentsen will give a focused update on primary chronic cold agglutinin disease, which should be distinguished from secondary cold agglutinin syndrome. He will highlight recent advances in understanding the clinical, histopathologic and immunologic aspects of the disease and the mechanism of red blood cell destruction that is entirely complement-mediated. An update on treatment will be presented, and the evolving development of complement-directed therapy will be reviewed.