Practical recommendations and consensus for the management of immune mediated hematologic diseases

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Background

In the last decade growing attention has been paid even to the so-called benign hematologic conditions, including immune mediated diseases. The latter encompass autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), autoimmune neutropenia (AIN), aplastic anemia (AA), and immune mediated disorders with thrombophilic or hemorragic symptoms (namely anti-phospholipid syndrome, thrombotic microangiopathies and acquired hemophilia). All these forms show a great clinical heterogeneity, ranging from mild easily manageable cases to life-threatening and fatal ones. Moreover, a plethora of novel laboratory tests and several new drugs are being developed and studied. Clinicians are therefore experiencing new challenges in the diagnosis and management of these diseases and practical recommendations and guidelines are lacking since available literature mainly relies on retrospective data often including small patient series. Furthermore, a deeper understanding of the immunologic mechanisms underlying the development of these rare and heterogeneous conditions will likely contribute to broaden the knowledge of disease pathogenesis in both primary and secondary forms and to develop novel target therapies.

In this special issue we will collect Experts’ opinions and recommendations regarding the diagnosis and management of immune mediated hematologic diseases. Furthermore, a focus on the mechanisms of immune mediated hematologic disease as well as an overview of hitherto underlying inborn errors of immunity will be presented. Particular attention will be paid to article dealing with the practical management of AIHA, ITP, AIN, AA, anti-phospholipid syndrome, thrombotic microangiopathies, acquired hemophilia. Specific settings, including the management of infectious or thrombotic complications, the workup of immune diseases secondary to tumors, primary immunodeficiencies, transplant or drugs, and the management of very rare clinical entities (i.e. Evans syndrome, moderate AA, large granular lymphocyte lymphoproliferative disorder etc.), will be explored. Furthermore, we will welcome manuscripts focusing on the immunologic mechanisms underlying immune mediated hematologic diseases and immunodeficiencies, including those dealing with novel markers of immune activation, insights in diseases pathogenesis, and predictors of response to therapy and outcome. All kind of manuscripts including expert opinions, consensus papers, literature reviews, and original articles with a focus on practical management of these diseases, as well as manuscripts dealing with old and new immunologic biomarkers, will be welcomed.

• Diagnosis and management of Evans’ syndrome (i.e. the association of AIHA, ITP, and/or AIN)
• Management of thrombotic and infectious risk in AIHA and ITP
• Diagnosis and management of hematologic disorders related to inborn errors of immunity
• Management of pregnancy in immune mediated hematologic conditions
• Management of AA (primary and secondary cases, moderate cases) and of AIN
• Diagnosis and management of autoimmune hemophilic and thrombophilic disorders
• Treatment choice in immune mediated hematologic conditions in the new drugs era and a focus on immunomodulatory properties of old and new drugs
• Immunological markers (like soluble mediators, molecular lesions, diseases associations etc) to be implemented in the understanding of diseases pathogenesis
• Immunological markers or techniques to be implemented in the diagnosis and management of immune mediated hematologic disorders
All types of articles will be accepted (see previous point), particularly original and research articles dealing addressing practical management and immunological mechanisms underlying the above mentioned conditions and expert consensus (i.e. Delphi surveys, etc.). If available, guidelines papers will also be welcomed.

Important note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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