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Histiocytic Disorders Amir H PHC15 12/06/2006 04:24PM Page 340 15 Histiocytic disorders Amir H. Shahlaee and Robert J. Arceci of phagocytosis of foreign material, antigen processing, and Introduction antigen presentation to lymphocytes. This system, recog- nized in part through the work of Metchnikoff, was originally The histiocytoses are a diverse group of hematologic dis- termed the reticuloendothelial system by Ludwig Aschoff orders defined by the pathologic infiltration of normal tissues in the early part of the twentieth century.3,4 The central cell by cells of the mononuclear phagocyte system (MPS). The of this system, the mononuclear phagocyte or histiocyte, heterogeneity of this family of disorders, a direct result of the represents a group of anatomically and functionally distinct biologic variability of the cells of the MPS and the tissues they cells arising from a common precursor, the hematopoietic inhabit, makes the study of these diseases one of the most stem cell. intriguing yet complex areas of modern hematology. Cells of the MPS have a wide range of morphologic, anatomic Advances in basic hematology and immunology over the and functional characteristics that make classification of this last two decades have significantly enhanced our under- system difficult. Our ability to identify and classify the cells standing of the histiocytic disorders. It is now accepted that of the MPS has advanced in parallel with developments in the pathogenic cells central to the development of the histio- basic hematology and immunology. As our knowledge of the cytoses arise from a common hematopoietic progenitor. More molecular biology regulating hematopoiesis has improved, specifically, the ability to molecularly identify the hemato- we have been able to identify specific characteristics that poietic cells has enabled us to classify the histiocytoses based have enabled us to classify the cells of the MPS. Mononuclear on the cellular basis of the disease and to define the natural phagocytes can be divided into two major classes, macro- history of these disorders.1,2 These pathologic cells phenotypic- phages and dendritic cells. This classification is based on ally resemble immature mononuclear phagocytes at specific (i) phagocytic and antigen-presenting abilities, (ii) morphologic stages of differentiation.77 and ultrastructural appearance, (iii) expression of common The Histiocyte Society, formed in 1985, has served as a enzymes, (iv) presence of common cell surface antigens, forum for enhanced collaboration between international and (v) common regulatory cytokine and transcription factor histiocytosis experts. Since its inception, the Histiocyte networks. Society has used the cellular based classification of the histio- Tissue macrophages are derived from bone marrow cytoses as a guideline for therapeutic studies, which have in hematopoietic precursors and can arise directly from circu- turn significantly advanced our ability to care for patients, lating peripheral blood monocytes. These cells enter different improve their outcomes and advance the scientific under- tissues and assume tissue-appropriate morphology based on standing of the histiocytoses. In this chapter we use the cellu- the local cytokine milieu. Once established in various tissues, lar classification of histiocytic disorders, as adopted by the macrophages typically do not have the ability to self-renew Histiocyte Society, to present each subgroup of this family of extensively except possibly under specialized microenviron- disorders, describe their natural history and present current ments such as in the lungs and pituitary. Tissue inflammation therapeutic approaches and outcomes. increases the influx of monocytes, which in turn give rise to macrophages with an immunologically activated phenotype. Inflammatory stimuli can also further enhance the local rep- Histiocytes and normal immune function lication of macrophages in cases of tissue injury.5,6 Regardless of location, macrophage growth and differentiation is a tightly The MPS is a system of cells whose primary function consists controlled process regulated by specific growth factors. 340 PHC15 12/06/2006 04:24PM Page 341 Histiocytic disorders Interleukin (IL)-3, IL-4, IL-13, granulocyte–macrophage often branch-like, cytoplasmic aberrations (from the Greek colony-stimulating factor (GM-CSF) and macrophage colony- word for tree, δενδρεον).15 Dendritic cells are primarily stimulating factor (M-CSF) are all stimulatory cytokines located in skin, mucosa, bone marrow, spleen, thymus and with a major role in macrophage development and differenti- lymph nodes. This group of cells also includes dendritic cells ation.6 M-CSF activity is especially essential for appropriate of the lymphoid follicle, the interdigitating dendritic cells of macrophage growth and differentiation. The M-CSF recep- the paracortical regions of lymph nodes, and Langerhans tor, a product of the c-fms protooncogene, is expressed by cells of the skin and other organs. Langerhans cells, originally most members of the MPS. When bound by M-CSF this identified by Paul Langerhans in the late 1800s, are mono- receptor dimerizes, leading to the activation of its kinase nuclear cells with little cytoplasmic vacuolization and a folded domain and initiation of downstream signaling pathways. or indented nucleus. Electron microscopic observation of The signaling cascade initiated by the binding of M-CSF to its Langerhans cells reveals the presence of Birbeck granules, a receptor is essential for the proliferation of progenitor cells of distinguishing characteristic of these cells. Birbeck granules, the monocyte–macrophage lineage, differentiation and their or X bodies, are rod-shaped, pentalaminar, cytoplasmic long-term survival. Information regarding the role of M-CSF inclusions, typically ending in a vesicular structure that arise primarily arises from studies of op/op knockout mice, which as a result of receptor-mediated endocytosis and are involved are deficient in the M-CSF receptor.7 Despite their severe in antigen processing.16 monocyte–macrophage deficiencies, op/op mice maintain In contrast to macrophages, dendritic cells are less normal levels of dendritic cells in their spleens and skin, thus phagocytic but play a central role in initiating primary T- demonstrating an alternative and M-CSF-independent path- lymphocyte antigen responses. The hallmark of dendritic way for their development.8,9 The GM-CSF receptor, also cells is their conversion from immature, peripherally located present on most members of the MPS, plays a critical role in sentinels capable of antigen capture and processing to normal cellular homeostasis, differentiation and function.8 mature immunostimulatory cells, a process called maturation. There is also a positive survival effect of stem cell factor (the This specific characteristic of dendritic cells, in combina- ligand for the c-KIT receptor) and FLT3 ligand and its cognate tion with their ability to migrate, provides a physical and receptor FLT3, as well as inhibitory roles for interferon (IFN)- functional link between peripheral tissues and secondary α/β, transforming growth factor (TGF)-β and leukocyte lymphoid organs where lymphocyte maturation takes inhibitory factor as critical determinants in macrophage place.17 Langerhans cells and the interdigitating dendritic development.6 The phenotypic and functional heterogeneity cells present antigens to stimulate primary T-lymphocyte of macrophages as a group is attributed to the tissue-specific responses. It is well established that the precursor to most effects of cytokine stimulation on the multipotent monocyte. dendritic cells is bone marrow derived but the biologic Pathologic changes in this cytokine milieu play important significance of this differentiation schema is still not com- roles in the pathogenesis of the histiocytoses.10,14 pletely defined as a significant amount of the data has Macrophages are ubiquitously distributed in the body been derived from in vitro studies.16 Other distinguishing and are heavily represented in mucosal tissues and other characteristics of mature dendritic cells include (i) high potential portals of entry for microorganisms, where they expression levels of class II major histocompatibility complex function in both innate and adaptive immunity. They have (MHC) antigens and costimulatory receptors, making them the capacity to phagocytose foreign organisms and release in- potent at antigen presentation, (ii) adenosine triphosphatase flammatory cytokines that in turn recruit other inflammation- and α-mannosidase expression and (iii) distinct paranuclear associated cell types. Macrophages also maintain the ability and cell surface-staining pattern with peanut agglutinin that to process and present antigenic portions of foreign organ- contrasts with the diffuse staining pattern demonstrated by isms to T lymphocytes, although not as effectively as den- macrophages.16,18 dritic cells. Tissue macrophages have significant functional The development of methods to generate monoclonal variability depending on their location within the body. antibodies in the 1970s and 1980s significantly enhanced our Peritoneal and soft tissue macrophages, Kupffer cells, foam knowledge of the MPS. The F4/80 antibody, a murine pan- cells, synovial cells, osteoclasts and microglial cells are all macrophage antibody, was one of the first well-recognized members of this class of cells and contribute to tissue markers of the MPS. This antibody recognizes members of a growth, repair and remodeling. A distinguishing feature
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