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1034 Part VII: Neutrophils, Eosinophils, Basophils, and Mast Cells Chapter 66: Disorders of Neutrophil Function 1035

Consider neutrophil Hemolytic anemia Howell-Jolly bodies Functional asplenia
G6PD deficiency 1. Initial evaluation
Abnormal granules • History, physical exam Thrombocytopenia,
Chédiak-Higashi partial albinism • Family history eczema Wiskott-Aldrich
syndrome • Leukocyte, platelet, syndrome
Abnormal granules reticulocyte, and Neutrophil counts
Consider specific Pelger-Hüet anomaly differential counts <1500 Neutropenia workup
granule deficiency • Bacterial cultures (bone marrow aspirate,
antineutrophil antibodies,
If normal serial CBCs to establish
cyclic neutropenia)
Hypogammaglobulinemia Decreased 2. Ig/complement workup
syndromes, e.g., X-linked immunoglobulins
agammaglobulinemia, • IgG, IgM, IgA levels
CVID • IgE level
• Antibody titers to vaccine
antigens, tetanus,
diphtheria, rubeola
Severe combined • Haemophilus influenzae,
immunodeficiency polysaccharide
(several variants), AIDS, • Delayed hypersensitivity
DiGeorge syndrome Cellular skin tests (Candida,
(thymic hypoplasia) immunodeficiencies tetanus, toxoid, STAT 3 mutation IgE; >2000 IU/mL;
mumps, tricophyton, AD hyper-IgE
Mucocutaneous streptokinase-
candidiasis streptodomase)
Wiskott-Aldrich syndrome • Chest and sinus radiographs
Ataxia-telangiectasia • IgG subclass levels for
IgG1, IgG2, IgG3, IgG4
• Total T cells, T-helper
Decreased cells, T-suppressor cells,
Hypocomplementemia complement and the ratio between
syndrome helpers and suppressors
• C3, C4, CH50 levels
If normal

–
– Abbreviated O 2
Chronic granulomatous Absent O 2 3. Phagocyte evaluation production Neutrophil G6PD
disease Abnormal NBT • NBT test deficiency
and DHR tests • DHR assay GSH pathway
• Chemotaxis assays
- Rebuck skin window LAD-1 and LAD-2
Only abnormal - In vitro assay with Chemotaxis Chédiak-Higashi
Complement deficiency, chemotaxis patient control sera syndrome specific
humoral defects Abnormal response granule deficiency
to activated control Rac-2 deficiency
serum
If normal
Absent CD11/CD18 by
decreased ingestion
4. Further phagocyte LAD-1
evaluation Decreased ingestion
Myeloperoxidase with control serum
Myeloperoxidase absent • Myeloperoxidase stain Neutrophil actin
deficiency • Flow cytometry to dysfunction
measure CD11/CD18 Decreased sLe x
surface glycoproteins expression by flow
on neutrophils LAD-2
Decreased ingestion • Quantitative ingestion
with patient’s serum Diminished adhesion
Opsonin defect assays (patient and to selectin ligand
control sera as opsonins) Rac-2 deficiency
• Flow cytometry to measure
selectin on neutrophils Failure to generate
–
• Rolling on L-selectin ligand O 2 when challenged LAD-3
with unopsonized
zymosam
Figure 66–8. Algorithm for the workup of patients with recurrent infections. AD, autosomal dominant; CBC, complete blood count; CVID, common
variable immunodeficiency; DHR, delayed hypersensitivity reaction; G6PD, glucose-6-phosphate dehydrogenase; GSH, glutathione; Ig, immunoglob-
ulin, LAD, leukocyte adhesion deficiency; NBT, nitroblue tetrazolium; sLe , sialyl Lewis X.
x

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