Abstract
Immune phenomena are increasingly reported in myeloid neoplasms, and include autoimmune cytopenias/diseases and immunodeficiency, either preceding or complicating acute myeloid leukemia, myelodysplastic syndromes (MDS), chronic myeloproliferative neoplasms, and bone marrow failure (BMF) syndromes. Autoimmunity and immunodeficiency are the two faces of a dysregulated immune tolerance and surveillance and may result, along with contributing environmental and genetic factors, in an increased incidence of both tumors and infections. The latter may fuel both autoimmunity and immune activation, triggering a vicious circle among infections, tumors and autoimmune phenomena. Additionally, alterations of the microbiota and of mesenchymal stem cells (MSCs) pinpoint to the importance of a permissive or hostile microenvironment for tumor growth. Finally, several therapies of myeloid neoplasms are aimed at increasing host immunity against the tumor, but at the price of increased autoimmune phenomena. In this review we will examine the epidemiological association of myeloid neoplasms with autoimmune diseases and immunodeficiencies, and the pivotal role of autoimmunity in the pathogenesis of MDS and BMF syndromes, including the paroxysmal nocturnal hemoglobinuria conundrum. Furthermore, we will briefly examine autoimmune complications following therapy of myeloid neoplasms, as well as the role of MSCs and microbiota in these settings.
Highlights
The immune system is broadly involved in maintaining homeostasis, either by fighting infectious agents or controlling tumor growth
Chronic/relapsing autoimmune hemolytic anemia (AIHA) show a possible evolution to ICUS, IDUS or bone marrow failure (BMF) syndromes over time, suggesting a shift from “peripheral” to “central” autoimmunity [27, 28, 53, 54]
The complexity of the issue is further increased by the heterogeneity of myeloid disorders that encompass truly malignant cells and more subtle diseases
Summary
The immune system is broadly involved in maintaining homeostasis, either by fighting infectious agents or controlling tumor growth. Immune Phenomena in Myeloid Neoplasms is the inability to efficiently eliminate infectious pathogens or neoplastic cells, and both may result in severe and lifethreatening diseases. The association of immunodeficiency with tumors is well known, together with the role of consequent chronic/relapsing infections that may fuel both autoimmunity and immune activation. The latter may be exaggerated, ineffective and potentially harmful, triggering a vicious circle among infections, tumors and autoimmune phenomena. This review will examine immune phenomena (autoimmunity and immunodeficiency) in myeloid neoplasms, including MDS, BMF syndromes, acute myeloid leukemia (AML), and chronic myeloproliferative neoplasms (MPN). We will focus on the several overlapping conditions, highlighting the continuous and mutual cross-talk between the immune effectors and the neoplastic cells (Figure 1)
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