Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ GENERAL CONSIDERATIONS ++ Initially, one should use appropriate normal neutrophil concentration values for certain ethnic groups in which neutrophil counts are significantly lower than persons of European ancestry (e.g., African ancestry, Yemeni Jewish ancestry). In this classification, diseases resulting from neutrophil abnormalities in which the neutrophil is either the only cell type affected or is the dominant cell type affected are considered (Table 31–1). Neutropenia or neutrophilia occurs as part of disorders that affect multiple blood cell lineages, (e.g., aplastic anemia [see Chap. 3], myelodysplastic syndrome [see Chap. 42], acute and chronic myelogenous leukemias [see Chaps. 46 and 47], chronic myeloproliferative diseases [see Chaps. 43, 44, and 48]). A pathophysiologic classification of neutrophil disorders has proved elusive because: — The low concentration of blood neutrophils in neutropenic states makes measuring the circulatory kinetics of autologous cells technically difficult. — The two compartments of neutrophils in the blood, the random disappearance of neutrophils from the circulation, the extremely short circulation time of neutrophils (t1/2 = ~6 hours), the absence of techniques to measure the size of the tissue neutrophil compartment, and the disappearance of neutrophils by apoptosis or excretion from the tissue compartment make multicompartment kinetic analysis difficult. Thus, the classification of neutrophil disorders is partly pathophysiologic and partly descriptive (see Table 31–1). ++Table Graphic Jump LocationTABLE 31–1CLASSIFICATION OF NEUTROPHIL DISORDERSView Table||Download (.pdf) TABLE 31–1 CLASSIFICATION OF NEUTROPHIL DISORDERS Quantitative disorders of neutrophils Neutropenia Decreased neutrophilic granulopoiesis Congenital severe neutropenias (Kostmann syndrome and related disorders) Reticular dysgenesis (congenital aleukocytosis) Neutropenia and exocrine pancreas dysfunction (Shwachman-Diamond syndrome) Neutropenia and immunoglobulin abnormality (e.g., hyper-IgM syndrome) Neutropenia and disordered cellular immunity (cartilage hair hypoplasia) Mental retardation, anomalies, and neutropenia (Cohen syndrome) X-linked cardioskeletal myopathy and neutropenia (Barth syndrome) Myelokathexis Warts, hypogammaglobulinemia, infection, myelokathexis (WHIM) syndrome Neonatal neutropenia and maternal hypertension Griscelli syndrome Glycogen storage disease 1b Hermansky-Pudlak syndrome Wiskott-Aldrich syndrome Chronic hypoplastic neutropenia (1) Drug-induced (2) Cyclic (3) Branched-chain aminoacidemia Acute hypoplastic neutropenia (1) Drug-induced (2) Infectious Chronic idiopathic neutropenia (1) Benign (a) Familial (b) Sporadic (2) Symptomatic Accelerated neutrophil destruction Alloimmune neonatal neutropenia Autoimmune neutropenia (1) Idiopathic (2) Drug-induced (3) Felty syndrome (4) Systemic lupus erythematosus (5) Other autoimmune diseases (6) Complement activation-induced neutropenia (7) Pure white cell aplasia Maldistribution of neutrophils Pseudoneutropenia Neutrophilia Increased neutrophilic granulopoiesis Hereditary neutrophilia Trisomy 13 or 18 Chronic idiopathic neutrophilia (1) Asplenia Neutrophilia or neutrophilic leukemoid reactions (1) Inflammation (2) Infection (3) Acute hemolysis or acute hemorrhage (4) Cancer, including granulocyte colony-stimulating factor (G-CSF)-secreting tumors (5) Drugs (e.g., glucocorticoids, lithium, granulocyte- or granulocyte-monocyte colony-stimulating factor, tumor necrosis factor-α) (6) Ethylene glycol exposure (7) Exercise Sweet syndrome Cigarette smoking Cardiopulmonary bypass Decreased neutrophil circulatory egress Drugs (e.g., glucocorticoids) Maldistribution of neutrophils Pseudoneutrophilia Qualitative disorders of neutrophils Defective adhesion of neutrophils Leukocyte adhesion deficiency Drug-induced Defective locomotion and chemotaxis Actin polymerization abnormalities Neonatal neutrophils Interleukin-2 administration Cardiopulmonary bypass Defective microbial killing Chronic granulomatous disease... 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