Hereditary White Cell Disorders

May-Hegglin Anomaly

This inherited disorder is associated with thrombocytopenia and giant platelets. Abnormal bleeding may be seen in a small number of affected individuals. Dohle bodies are seen in the cytoplasm of neutrophils and are larger than the Dohle bodies seen in neutrophils responding to infections or inflammation.

Alder's Anomaly (Alder-Reilly Anomaly)

This rare genetic disorder is associated with the presence of coarse dark granules in neutrophils, lymphocytes, monocytes, eosinophils, and basophils (Fig. 10.8). This granulation is thought to consist of lipid depositions in the cytoplasm as a result of decreased mucopolysaccharide production.7 Prominent deposition of granules in every cell line is a differentiating feature between this condition and toxic granulation, which appears in clusters only in neutrophils and monocytes.

Pelger-Huët Anomaly

This fairly common inherited disorder shows hyposeg-mentation of the nucleus of segmented neutrophils. In heterozygotes, the nucleus is seen as peanut shaped, dumb-bell shaped, or pince-nez shaped (Fig. 10.9). In homozygotes, the nucleus is spherical with no lobes and prominent nuclear clumping. Initially, when observing cells with the Pelger-Huët anomaly, they may appear as bands or metamyelocytes. When considering these cells in a peripheral smear, it is important

Pseudo Pelger Huet Cells
Figure 10.9 Pelger-Huet. Note spherocyte (at arrow) and the typical bilobed appearance of Pelger-Huet cells.

to make two judgments. Is the hyposegmentation seen in the majority of neutrophils? Is the nuclear content mature? Even experienced morphologists have misidentified these cells as bands or metamyelocytes, greatly skewing the peripheral smear results. In a true Pelger-Huët anomaly, almost 70% to 95% of the neutrophils will show hyposegmentation. The cells, however, will be functional neutrophils. There are a number of conditions in which the neutrophils may present with a pseudo-Pelger-Huët look, such as leukemias, myeloproliferative disorders, and severe infections.

Chediak-Higashi Syndrome

This is a rare autosomal disorder of neutrophilic granules. Neutrophils in these individuals show giant gray-green cytoplasmic granules (Fig. 10.10). Lymphocytes

Bony Deformities Chediak Higashi

Figure 10.10 Chediak-Higashi anomaly. Note large gray-green granules in the cytoplasm.

Figure 10.8 Alder's anomaly. Note deep granulation.

Figure 10.10 Chediak-Higashi anomaly. Note large gray-green granules in the cytoplasm.

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