Chapter 62: Classification and Clinical Manifestations of Neutrophil Disorders

I. Quantitative Disorders of Neutrophils

 A. Neutropenia^16–18

  1. Decreased neutrophilic granulopoiesis

   a. Congenital severe neutropenias (Kostmann syndrome and related disorders)^19–21

   b. Reticular dysgenesis (congenital aleukocytosis)^22,23

   c. Neutropenia and exocrine pancreas dysfunction (Shwachman-Diamond syndrome)^24,25

   d. Neutropenia and immunoglobulin abnormality (eg, hyperimmunoglobulin M syndrome)^26–28

   e. Neutropenia and disordered cellular immunity (cartilage hair hypoplasia)^29–31

   f. Mental retardation, anomalies, and neutropenia (Cohen syndrome)^32–34

   g. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome)^35–38

   h. Myelokathexis^39,40

   i. Warts, hypogammaglobulinemia, infection, myelokathexis (WHIM)syndrome^41,42

   j. Neonatal neutropenia and maternal hypertension^43,44

   k. Griscelli syndrome⁴⁵

   l. Glycogen storage disease 1b⁴⁶

   m. Hermansky-Pudlak syndrome 2^47,48

   n. Wiskott-Aldrich syndrome⁴⁹

   o. Chronic hypoplastic neutropenia

    (1) Drug-induced^50–53

    (2) Cyclic^54–56

    (3) Branched-chain aminoacidemia⁵⁷

   p. Acute hypoplastic neutropenia

    (1) Drug induced^58–61

    (2) Infectious⁶²

   q. Chronic idiopathic neutropenia

    (1) Benign^63–65

     (a) Familial⁶³

     (b) Sporadic⁶³

    (2) Symptomatic^64–65

  2. Accelerated neutrophil destruction

   a. Alloimmune neonatal neutropenia^66–68

   b. Autoimmune neutropenia^69–71

    (1) Idiopathic⁷²

    (2) Drug induced⁷³

    (3) Felty syndrome^74–76

    (4) Systemic lupus erythematosus^77,78

    (5) Other autoimmune diseases^79–84

    (6) Complement activation-induced neutropenia⁸⁵

    (7) Pure white cell aplasia^{84,86–88...}