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Chapter 7
Myeloid disorders
Introduction
The term "myeloid" derives from the Greek myelos, meaning "marrow." Sometimes the term "myeloid" is used to describe hematological 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, dendritic cells, and mast cells. The myeloproliferative disorders (ie,
Granulocytes: neutrophils, eosinophils, and basophils
Granulopoiesis Neutrophil release from the bone marrow Neutrophil extravasation
Monocytes and tissue histiocytes
Dendritic antigen-presenting cells
Mast cells
Neutrophilia
Neutropenia
Congenital or inherited 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 Familial Mediterranean fever
Monocytosis
Disorders of histiocytes and dendritic cells
Hemophagocytic lymphohistiocytosis Diagnosis and prognosis Treatment Langerhans cell histiocytosis Diagnosis Treatment
Mastocytosis
Diagnosis of CM and SM Treatment of CM and SM | |||||||||||||||||||||||||||||||||||||||||||||||||||