Congenital and acquired disorders of macrophages and histiocytes:
Gespeichert in:
| Weitere Verfasser: | |
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| Format: | Buch |
| Sprache: | English |
| Veröffentlicht: |
Philadelphia, Pa.
Elsevier
2015
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| Schriftenreihe: | Hematology, oncology clinics of North America
29,5 |
| Online-Zugang: | Inhaltsverzeichnis Inhaltsverzeichnis |
| Beschreibung: | XV S., S. 799 - 970 Ill., graph. Darst. |
| ISBN: | 9780323400886 |
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Datensatz im Suchindex
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| adam_text | Titel: Congenital and acquired disorders of macrophages and histiocytes
Autor: Berliner, Nancy
Jahr: 2015
Congenital and Acquired Disorders of Macrophages and Histiocytes
Contents
Dedication xj
Barrett J. Rollins, Carl E. Allen, Matthew Collin, Ronald Jaffe,
Jennifer L. Picarsic, and Carlos Rodriguez-Galindo
Preface: Congenital and Acquired Disorders of Macrophages and Histiocytes xiii
Nancy Berliner and Barrett J. Rollins
Nosology and Pathology of Langerhans Cell Histiocytosis 799
Jennifer Picarsic and Ronald Jaffe
The classification of the histiocytoses has evolved based on new under-
standing of the cell of origin as a bone marrow precursor. Although the
pathologic features of the histiocytoses have not changed per se, molec-
ular genetic information now needs to be integrated into the diagnosis. The
basic lesions of the most common histiocytoses, their patterns in different
sites, and ancillary diagnostics are now just one part of the classification.
As more is understood about the cell of origin and molecular biology of the
histiocytoses, future classifications will be refined.
Cell(s) of Origin of Langerhans Cell Histiocytosis 825
Matthew Collin, Venetia Bigley, Kenneth L. McClain, and Carl E. Allen
Langerhans cell histiocytosis (LCH) is a heterogeneous disease character-
ized by common histology of inflammatory lesions containing Langerin+
(CD207) histiocytes. Emerging data support a model in which MAPK
activation in self-renewing hematopoietic progenitors may drive dissemi-
nated high-risk disease, whereas MAPK activation in more differentiated
committed myeloid populations may induce low-risk LCH. The heteroge-
neous clinical manifestations with shared histology may represent the final
common pathway of an acquired defect of differentiation, initiated at more
than one point. Implications of this model include re-definition of LCH as a
myeloid neoplasia and re-focusing therapeutic strategies on the cells and
lineages of origin.
Genomic Alterations in Langerhans Cell Histiocytosis 839
Barrett J. Rollins
The discovery of recurrent somatic genomic alterations in Langerhans cell
histiocytosis (LCH) has led to a new understanding of LCH as a clonal
neoplastic disorder. Most of the abnormalities described to date affect
the RAS/RAF/MEK/extracellular-signal-regulated kinase (ERK) pathway:
more than 50% of LCH cases carry activating mutations in BRAF, whereas
another 10% to 28% carry activating mutations of MAP2K1, which en-
codes MEK1. The pathogenetic importance of these mutations has been
confirmed by reports of significant clinical responses to RAF inhibitors.
viii Contents
Clinical Characteristics and Treatment of Langerhans Cell Histiocytosis 853
Chalinee Monsereenusorn and Carlos Rodriguez-Galindo
Langerhans cell histiocytosis (LCH) is a disease caused by clonal prolifer-
ation of CD1 a+/CD207+ cells that is characterized by a spectrum of vary-
ing degrees of organ involvement and dysfunction. Treatment of LCH is
risk adapted; patients with single lesions may respond well to local treat-
ment, whereas patients with multi-system disease and risk-organ involve-
ment require more intensive therapy. Although survival for patients without
organ dysfunction is excellent, mortality rates for patients with organ
dysfunction may reach 30% to 40%. For patients with low-risk disease,
although cure is almost universal, disease reactivation rates are in excess
of 30%.
Strategies for the Prevention of Central Nervous System Complications in
Patients with Langerhans Cell Histiocytosis: The Problem of Neurodegenerative
Syndrome 875
Shinsaku Imashuku and Robert J. Arcecif
Diseases of the central nervous system (CNS) are common in patients with
Langerhans cell histiocytosis (LCH). Besides active LCH lesions, neurode-
generative (ND) lesions of the cerebellum and/or basal ganglia may occur
as late sequelae of LCH. While the etiology of this ND disease remains un-
clear, biomarkers in cerebrospinal fluid (CSF) may reflect the activity of
CNS disease in these patients. However, no well-planned CSF studies
have yet been performed in patients at high risk for ND-CNS-LCH. Poten-
tial parallels with other neuroinflammatory/neurodegenerative diseases
suggest the utility of examining these other disorders in establishing stra-
tegies for the prevention and/or treatment of ND-CNS-LCH.
Pathogenesis of Hemophagocytic Lymphohistiocytosis 895
Alexandra H. Filipovich and Shanmuganathan Chandrakasan
Hemophagocytic Lymphohistiocytosis (HLH), an inherited life-threatening
inflammatory disorder, has gained growing recognition not only in children
but also increasingly in adults over the past 2 decades. HLH involves
inborn defects in lymphocytes, which normally mediate control of infec-
tious and inflammatory conditions within the immune system and in other
tissues. In the context of inherited defects in cytotoxic cells and other im-
mune cells, the disorder is classified as familial or primary HLH. Secondary
HLH occurs in the settings of infections or underlying rheumatologic disor-
ders. Secondary HLH also accompanies some lymphoid malignancies.
Familial Hemophagocytic Lymphohistiocytosis 903
Barbara Degar
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare heritable dis-
order of immune regulation that is typically characterized by sudden onset
of severe systemic illness. Functional impairment or absence of 1 or more
of several proteins that participate in lymphocyte cytotoxicity underlies the
disease. Although FHL usually presents in infancy, age of onset is variable
and dependent on genetic and environmental factors. Initial treatment
Contents ix
consists of immune suppression, whereas definitive treatment requires
hematopoietic cell transplantation.
Hemophagocytic Lymphohistiocytosis in Adults 915
Meghan Campo and Nancy Berliner
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal
syndrome of pathologic immune dysregulation characterized by clinical
signs and symptoms of extreme inflammation. HLH can occur as a genetic
or sporadic disorder and, though seen as an inherited condition affecting
primarily a pediatric population, can occur at any age and can be encoun-
tered in association with a variety of underlying diseases. Clinically,
the syndrome, whether genetic or acquired, is characterized by fever,
hepatosplenomegaly, cytopenias, and activated macrophages in hemato-
poietic organs. Therapy centers on suppression of this hyperinflammatory
state with cytotoxic, immunosuppressive therapy and treatment of any
existing HLH triggers.
Macrophage Activation Syndrome 927
Angelo Ravelli, Sergio Davi, Francesca Minoia, Alberto Martini, and
Randy Q. Cron
Macrophage activation syndrome (MAS) is a potentially life-threatening
complication of rheumatic disorders that occurs most commonly in sys-
temic juvenile idiopathic arthritis. In recent years, there have been several
advances in the understanding of the pathophysiology of MAS. Further-
more, new classification criteria have been developed. Although the place
of cytokine blockers in the management of MAS is still unclear, interleukin-1
inhibitors represent a promising adjunctive therapy, particularly in refrac-
tory cases.
The Role of Hematopoietic Stem Cell Transplantation in Treatment of
Hemophagocytic Lymphohistiocytosis 943
Sarah Nikiforow
The role of reduced-intensity allogeneic hematopoietic stem cell trans-
plantation (HSCT) from a variety of donor sources in improving survival
for children with familial hemophagocytic lymphohistiocytosis (HLH) is
well-documented. The heterogeneity of adult-onset HLH has complicated
evaluation of initial therapy and of HSCT as definitive treatment. Therapy
for adults with HLH is often individualized, but institutions are now gener-
ating algorithms that include HSCT based on growing experience. Consol-
idation of these data is needed to optimize management of the growing
number of adults recognized to have HLH and to achieve dramatic
improvements in survival.
Index
961
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| spelling | Congenital and acquired disorders of macrophages and histiocytes ed. Nancy Berliner ... Philadelphia, Pa. Elsevier 2015 XV S., S. 799 - 970 Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Hematology, oncology clinics of North America 29,5 Clinics review articles Berliner, Nancy (DE-588)1013160274 edt Hematology, oncology clinics of North America 29,5 (DE-604)BV000625446 29,5 http://digitool.hbz-nrw.de:1801/webclient/DeliveryManager?pid=6547506&custom_att_2=simple_viewer Congenital and acquired disorders of macrophages and histiocytes Inhaltsverzeichnis HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=028620112&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
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| title | Congenital and acquired disorders of macrophages and histiocytes |
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| title_exact_search | Congenital and acquired disorders of macrophages and histiocytes |
| title_full | Congenital and acquired disorders of macrophages and histiocytes ed. Nancy Berliner ... |
| title_fullStr | Congenital and acquired disorders of macrophages and histiocytes ed. Nancy Berliner ... |
| title_full_unstemmed | Congenital and acquired disorders of macrophages and histiocytes ed. Nancy Berliner ... |
| title_short | Congenital and acquired disorders of macrophages and histiocytes |
| title_sort | congenital and acquired disorders of macrophages and histiocytes |
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