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Rare macrophage diseases in adults
Affiliations- Medical Faculty, University of Rzeszow, Rzeszow, Poland
- Hematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Sweden
- Department of Clinical Pathology and Cytology, Karolinska University Hospital Huddinge, Stockholm, Sweden
open access
Abstract
Diseases of macrophages are rare entities characterized by the accumulation of macrophages, dendritic cells or monocyte-derived cells in various tissues and organs. This article focuses on macrophage disorders which can be present in adult patients. The review highlights pathogenesis, signs and symptoms, diagnostic criteria and principles of therapy in the most frequent macrophage diseases in adults: hemophagocytic lymphohistiocytosis, Rosai-Dorfman disease, Gaucher disease, Niemann-Pick disease, and Langerhans cell histiocytosis. Since macrophage disorders can be encountered by various medical specialists, basic knowledge of these entities and their diagnostic criteria should be familiar to all physicians, including hematologists.
Abstract
Diseases of macrophages are rare entities characterized by the accumulation of macrophages, dendritic cells or monocyte-derived cells in various tissues and organs. This article focuses on macrophage disorders which can be present in adult patients. The review highlights pathogenesis, signs and symptoms, diagnostic criteria and principles of therapy in the most frequent macrophage diseases in adults: hemophagocytic lymphohistiocytosis, Rosai-Dorfman disease, Gaucher disease, Niemann-Pick disease, and Langerhans cell histiocytosis. Since macrophage disorders can be encountered by various medical specialists, basic knowledge of these entities and their diagnostic criteria should be familiar to all physicians, including hematologists.
Keywords
Hemophagocytic lymphohistiocytosis; Rosai-Dorfman disease; Gaucher disease; Niemann-Pick disease; Langerhans cell histiocytosis
About this articleTitle
Rare macrophage diseases in adults
Journal
Issue
Pages
146-154
Published online
2016-04-01
Page views
106
Article views/downloads
598
DOI
10.1016/j.achaem.2016.04.006
Bibliographic record
Acta Haematol Pol 2016;47(2):146-154.
Keywords
Hemophagocytic lymphohistiocytosis
Rosai-Dorfman disease
Gaucher disease
Niemann-Pick disease
Langerhans cell histiocytosis
Authors
Maciej Machaczka
Monika Klimkowska