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Chapter 13
Myeloid disorders
The term "myeloid" derives from the Greek myelos, meaning "marrow." Sometimes the term "myeloid" is used to describe hematologic conditions or diseases not involving the lymphoid tissues or lymphocytes. "Myeloid" is also used to describe disorders primarily involving granulocytes (neutrophils, eosinophils, or basophils) and monocytes. Tissue macrophages (histiocytes), Langerhans cells, and interdigitating dendritic cells also arise from monocytic progenitors or precursors. Other important immunoregulatory dendritic cell types and mast cells derive from marrow progenitors that are distinct from myeloid and monocytic progenitors. This chapter covers disorders of granulocytes, monocytes, histiocytes, and dendritic cells. The myelo proliferative disorders (ie, the
Granulocytes: neutrophils, eosinophils, and basophils
Granulopoiesis Neutrophil release Neutrophil clearance Neutrophil extravasation
Monocytes and tissue histiocytes
Dendritic cells
Neutrophilia
Neutropenia
Severe congenital neutropenia (Kostmann syndrome) Cyclic neutropenia Shwachman-Diamond syndrome WHIM syndrome Chédiak-Higashi syndrome Neonatal alloimmune neutropenia Primary autoimmune neutropenia Secondary autoimmune neutropenia Large granular lymphocyte leukemia Nonimmune chronic idiopathic neutropenia Neutropenia due to idiosyncratic drug reactions Chemotherapy-induced neutropenia
Disorders of neutrophil function
Myeloperoxidase deficiency Leukocyte adhesion deficiency Hyperimmunoglobulin E syndrome Neutrophil-specific granule deficiency Chronic granulomatous disease Autoinflammatory diseases
Monocytosis
Monocytopenia
Disorders of histiocytes and DCs
Hemophagocytic lymphohistiocytosis Langerhans cell histiocytosis Non–Langerhans cell histiocytoses
Lysosomal storage diseases
Gaucher disease Niemann-Pick disease | ||||||||||||||||||||||||||||||||||||||||||||||||||