Figure 1.

Figure 1.

Examples of granules in polymorphonuclear neutrophils, platelet-dense bodies, and pigment in hair shafts for controls, classic Chediak-Higashi syndrome (CHS), and atypical CHS

a. Whole-mount electron microscopy (EM) of control platelets shows several dense bodies per platelet (arrows).

b. Some CHS platelets have no dense bodies (asterisk) and others have irregular electron-dense granules (arrows).

c. Normal control polymorphonuclear neutrophils (PMNs) contain numerous small cytoplasmic granules.

d. The blood smear derived from an individual with classic severe CHS shows PMNs that contain enlarged intracytoplasmic granules.

e. PMNs from an adult-onset, mildly affected individual with atypical CHS contain many granules that are larger than normal but smaller than those of the individual with classic CHS seen in 1d.

f. Control hair shows pigment that is evenly distributed throughout the shaft.

g. Hair of an individual with classic CHS shows an irregular distribution of large and small pigment clumps.

From: Chediak-Higashi Syndrome

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