Chapter 64: Classification and Clinical Manifestations of Neutrophil Disorders

I. Quantitative Disorders of Neutrophils

 A. Neutropenia^12,13

  1. Decreased neutrophilic granulopoiesis

   a. Congenital severe neutropenias (Kostmann syndrome and related disorders)^14,15

   b. Reticular dysgenesis (congenital aleukocytosis)^16,17

   c. Neutropenia and exocrine pancreas dysfunction (Shwachman-Diamond syndrome)^13,18

   d. Neutropenia and immunoglobulin abnormality (e.g., hyperimmunoglobulin M syndrome)^19,20,21

   e. Neutropenia and disordered cellular immunity (cartilage hair hypoplasia)^22,23

   f. Mental retardation, anomalies, and neutropenia (Cohen syndrome)^24,25

   g. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome)^26,27

   h. Myelokathexis^28,29

   i. Warts, hypogammaglobulinemia, infection, myelokathexis (WHIM) syndrome^30,31

   j. Neonatal neutropenia and maternal hypertension^32,33

   k. Griscelli syndrome³⁴

   l. Glycogen storage disease 1b³⁵

   m. Hermansky-Pudlak syndrome 2^36,37

   n. Wiskott-Aldrich syndrome³⁸

   o. Chronic hypoplastic neutropenia

    (1) Drug-induced^39,40,41,42

    (2) Cyclic^43,44

    (3) Branched-chain aminoacidemia⁴⁵

   p. Acute hypoplastic neutropenia

    (1) Drug-induced^39,46,47

    (2) Infectious⁴⁸

   q. Chronic idiopathic neutropenia

    (1) Benign

     (a) Familial⁴⁹

     (b) Sporadic⁵⁰

    (2) Symptomatic^51,52,53

  2. Accelerated neutrophil destruction

    a. Alloimmune neonatal neutropenia^54,55,56

    b. Autoimmune neutropenia^57,58,59

     (1) Idiopathic⁵⁹

     (2) Drug-induced^59,60

     (3) Felty syndrome^61,62,63

     (4) Systemic lupus erythematosus^64,65

     (5) Other autoimmune diseases^{66,67,68,69,70,71}

     (6) Complement activation-induced neutropenia⁷²

     (7) Pure white cell aplasia^71,73,74,75

   3. Maldistribution of neutrophils

    a. Pseudoneutropenia^76,77,78

 B. Neutrophilia

  1. Increased neutrophilic granulopoiesis

   a. Hereditary neutrophilia⁷⁹

   b. Trisomy 13 or 18⁸⁰

   c. Chronic idiopathic neutrophilia⁸¹

    (1) Asplenia⁸²

   d. Neutrophilia or neutrophilic leukemoid reactions

    (1) Inflammation^83,84

    (2) Infection^83,84,85

    (3) Acute hemolysis or acute hemorrhage⁸³

    (4) Cancer, including granulocyte colony-stimulating factor (G-CSF)-secreting tumors^86,87,88,89

    (5) Drugs (e.g., glucocorticoids, lithium, ...