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Chapter 29: Classification and Clinical Manifestations of Neutrophil Disorders

GENERAL CONSIDERATIONS

  • One should use appropriate normal neutrophil concentration values for certain ethnic groups in which neutrophil counts are significantly lower than persons of European ancestry (eg, 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 29–1).

  • Neutropenia or neutrophilia occurs as part of disorders that affect multiple blood cell lineages (eg, aplastic anemia [see Chap. 3], myelodysplastic syndrome [see Chap. 44], acute and chronic myelogenous leukemias [see Chaps. 45 and 46], chronic myeloproliferative diseases [see Chaps. 41, 42, and 47]).

  • A strict 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 facile techniques to measure the size of the tissue neutrophil compartment, and the disappearance of neutrophils by apoptosis or gastrointestinal excretion from the tissue compartment make multicompartment kinetic analysis difficult.

  • Thus, the classification of neutrophil disorders is partly pathophysiologic and partly descriptive (see Table 29–1).

TABLE 29–1CLASSIFICATION OF NEUTROPHIL DISORDERS
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TABLE 29–1 CLASSIFICATION OF NEUTROPHIL DISORDERS

  1. Quantitative Disorders of Neutrophils

    1. Neutropenia

      1. Decreased neutrophilic granulopoiesis

        1. Congenital severe neutropenias (Kostmann syndrome and related disorders)

        2. Reticular dysgenesis (congenital aleukocytosis)

        3. Neutropenia and exocrine pancreas dysfunction (Shwachman-Diamond syndrome)

        4. Neutropenia and immunoglobulin abnormality (eg, hyperimmunoglobulin M syndrome)

        5. Neutropenia and disordered cellular immunity (cartilage hair hypoplasia)

        6. Mental retardation, anomalies, and neutropenia (Cohen syndrome)

        7. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome)

        8. Myelokathexis

        9. Warts, hypogammaglobulinemia, infection, myelokathexis (WHIM) syndrome

        10. Neonatal neutropenia and maternal hypertension

        11. Griscelli syndrome

        12. Glycogen storage disease 1b

        13. Hermansky-Pudlak syndrome 2

        14. Wiskott-Aldrich syndrome

        15. Chronic hypoplastic neutropenia

          • (1) Drug-induced

          • (2) Cyclic

          • (3) Branched-chain aminoacidemia

        16. Acute hypoplastic neutropenia

          • (1) Drug-induced

          • (2) Infectious

        17. Chronic idiopathic neutropenia

          • (1) Benign

            • (a) Familial

            • (b) Sporadic

          • (2) Symptomatic

      2. Accelerated neutrophil destruction

        1. Alloimmune neonatal neutropenia

        2. 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

      3. Maldistribution of neutrophils

        1. Pseudoneutropenia

    2. Neutrophilia

      1. Increased neutrophilic granulopoiesis

        1. Hereditary neutrophilia

        2. Trisomy 13 or 18

        3. Chronic idiopathic neutrophilia

          • (1) Asplenia

        4. 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 (eg, glucocorticoids, lithium, granulocyte- or granulocyte-monocyte colony-stimulating factor, tumor necrosis factor-α)

          • (6) Ethylene glycol exposure

          • (7) Exercise

        5. Sweet syndrome

        6. Cigarette smoking

        7. Cardiopulmonary bypass

      2. Decreased neutrophil circulatory egress

        1. Drugs (eg, glucocorticoids)

      3. Maldistribution of neutrophils

        1. Pseudoneutrophilia

  2. Qualitative Disorders of Neutrophils

    1. Defective adhesion of neutrophils

      1. Leukocyte adhesion deficiency

      2. Drug-induced

    2. Defective locomotion and chemotaxis

      1. Actin polymerization abnormalities

      2. Neonatal neutrophils

      3. Interleukin-2 administration

      4. Cardiopulmonary bypass

    3. Defective microbial ...

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