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Chapter 29: Classification and Clinical Manifestations of Neutrophil Disorders

GENERAL CONSIDERATIONS

  • Normal neutrophil concentration values are significantly lower for certain ethnic groups (eg, African ancestry, Yemeni Jewish ancestry) than for persons of European ancestry. This difference is an important distinction to avoid unnecessary evaluations in these individuals. Men of African ancestry may have a “normal” absolute neutrophil count of 1.3 to 6.6 × 109/L.

  • 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. 45], acute and chronic myelogenous leukemias [see Chaps. 46 and 47], chronic myeloproliferative diseases [see Chaps. 42, 43, and 48]).

  • 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 circulating compartment measured by the absolute neutrophil count and the marginated neutrophil compartment sequestered in small vessel beds and not counted in the neutrophil count), 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

            1. Familial

            2. Sporadic

          • (2) Symptomatic

        18. 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

        19. Maldistribution of neutrophils

          1. Pseudoneutropenia

        20. 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) ...

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