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984 Part VII: Neutrophils, Eosinophils, Basophils, and Mast Cells CHAPTER 64: Classification and Clinical Manifestations of Neutrophil Disorders 985

TABLE 64–1. Classification of Neutrophil Disorders
I. Quantitative Disorders of Neutrophils 2. Accelerated neutrophil destruction II. Qualitative Disorders of Neutrophils
A. Neutropenia 12,13 a. Alloimmune neonatal A. Defective adhesion of neutrophils
1. Decreased neutrophilic neutropenia 54–56 1. Leukocyte adhesion deficiency 104,105
granulopoiesis b. Autoimmune neutropenia 57–59 2. Drug-induced 106
a. Congenital severe neutrope- (1) Idiopathic 59 B. Defective locomotion and chemotaxis
nias (Kostmann syndrome and (2) Drug-induced 59,60
related disorders) 14,15, (3) Felty syndrome 61–63 1. Actin polymerization
abnormalities
107–110
b. Reticular dysgenesis (congenital 111
aleukocytosis) 16,17 (4) Systemic lupus 64,65 2. Neonatal neutrophils
erythematosus
c. Neutropenia and exocrine 3. Interleukin-2 administration 112
pancreas dysfunction (Shwach- (5) Other autoimmune 4. Cardiopulmonary bypass 101
66–71
man-Diamond syndrome) 13,18 diseases C. Defective microbial killing
d. Neutropenia and immunoglob- (6) Complement activation- 1. Chronic granulomatous
72
ulin abnormality (e.g., hyperim- induced neutropenia disease 113,114
munoglobulin M syndrome) 19–21 (7) Pure white cell aplasia 71,73–75 2. RAC-2 deficiency 115,116
e. Neutropenia and disordered 3. Maldistribution of neutrophils 3. Myeloperoxidase deficiency 117,118
cellular immunity (cartilage hair a. Pseudoneutropenia 76–78
hypoplasia) 22,23 B. Neutrophilia 4. Hyperimmunoglobulin E (Job)
119,120
f. Mental retardation, anoma- 1. Increased neutrophilic granulopoiesis syndrome
lies, and neutropenia (Cohen 5. Glucose-6-phosphate dehydroge-
syndrome) 24,25 a. Hereditary neutrophilia 79 nase deficiency 121,122
g. X-linked cardioskeletal myop- b. Trisomy 13 or 18 80 6. Extensive burns 123,124
athy and neutropenia (Barth c. Chronic idiopathic neutrophilia 81 7. Glycogen storage disease Ib 125,126
syndrome) 26,27 (1) Asplenia 82 8. Ethanol toxicity 127,128
h. Myelokathexis 28,29 d. Neutrophilia or neutrophilic leu- 9. End-stage renal disease 129
i. Warts, hypogammaglobuline- kemoid reactions 10. Diabetes mellitus 130
mia, infection, myelokathexis (1) Inflammation 83,84
(WHIM) syndrome 30,31 83–85 D. Abnormal structure of the nucleus or of
j. Neonatal neutropenia and (2) Infection an organelle 131
maternal hypertension 32,33 (3) Acute hemolysis or acute 1. Hereditary macropolycytes
83
k. Griscelli syndrome 34 hemorrhage 2. Hereditary hypersegmentation 135
l. Glycogen storage disease 1b 35 (4) Cancer, including granulocyte 3. Specific granule deficiency 136–138
colony-stimulating factor
m. Hermansky-Pudlak syndrome (G-CSF)-secreting tumors 86–89 4. Pelger-Huët anomaly 139,140
2 36,37 (5) Drugs (e.g., glucocorticoids, 5. Alder-Reilly anomaly 141
n. Wiskott-Aldrich syndrome 38 lithium, granulocyte- or 6. May-Hegglin anomaly 142–144
o. Chronic hypoplastic granulocyte-monocyte col- 7. Chédiak-Higashi disease 145,146
neutropenia ony-stimulating factor, tumor III. Neutrophil-Induced Vascular or Tissue
83,90–94
(1) Drug-induced 39–42 necrosis factor-α) 83 Damage 147–149
(2) Cyclic 43,44 (6) Ethylene glycol exposure A. Pulmonary disease 150–155
95,96
(3) Branched-chain (7) Exercise B. Transfusion-related lung injury 156,157
aminoacidemia 45 e. Sweet syndrome 97,98 C. Renal disease 158,159
p. Acute hypoplastic neutropenia f. Cigarette smoking 99,100 D. Arterial occlusion 160,161
(1) Drug-induced 39,46,47 g. Cardiopulmonary bypass 101 E. Venous occlusion 162
(2) Infectious 48 2. Decreased neutrophil circulatory F. Myocardial infarction 157–163,167
q. Chronic idiopathic neutropenia egress 102 G. Ventricular function 164–168
(1) Benign a. Drugs (e.g., glucocorticoids) H. Stroke 157,169
(a) Familial 49 3. Maldistribution of neutrophils I. Neoplasia 170–172
103
(b) Sporadic 50 a. Pseudoneutrophilia J. Sickle cell vasoocclusive crisis 157,173
(2) Symptomatic 51–53

RAC-2, RAS-Related C3 botulinum toxin substrate 2.

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