Lecture 2 Leukocyte Non-malignant Hereditary Disorders Chapter 26

Question 1

What are the characteristics of May-Hegglin Anomaly?

Answer 1

Leukopenia
Thrombocytopenia
Giant Platelets
Dohle bodies (most often in neutrophils, but also in monocytes, basophils & eosinophils).

Question 2

What do the dohle bodies look like in May-Hegglin anomaly?

Answer 2

Dohle bodies in May-Hegglin may appear spindle shaped, more randomly distriubted than when there is dohle bodies due to an infection.

Dohle bodies look like basophilic inclusions 2-5 um in dia.

Question 3

What differentiates Pseudo Pelger-Huet from Pelger-Huet?

Answer 3

Pseudo Pelger-Huet shows a hypograndular cytoplasm whereas true Pelger-Huet is not.

Question 4

In what conditions is pseudo Pelger-Huet seen?

Answer 4

Pseudo Pelger-Huet is found in:
- myeloproliferative
- myelocysplastic
- acute leukemias
- acute infections, drug treatments, drug sensitivities and burns.

Question 5

What is the clinical implications of Pelger-Huet Anomaly?

Answer 5

Pelger-Huet cells are cytochemically and functionally normal.

Important to recognize Pelger-Huet & report to Dr.

Question 6

What are two types of nonmalignant hereditary disorders of Leukocytes?

Answer 6

1. Nuclear abnormalities (Pelger-Huet, Hereditary Neutrophil Hypersegmentation)
2. Cytoplasmic abnormalities (May-Hegglin Anomaly, Alder-Reilly Anomaly, Chediak-Higashi Syndrom).

Question 7

What is the result on the morphology of Pelger-Huet Anomaly?

Answer 7

Pelger-Huet Morphology:
1. Hyposegmentation of the granulocyte nucleus with increased density & increased coarseness of chromatin.
a. Heterozygous form - 2 symmetric round lobes connected by fine filament or fails to segment –> peanut or dumbell shape; clumping of chromatin overly mature for shape of nucleus.
b. Homozygous - rare form, round forms predominate.

Question 8

What is the morphology seen in hereditary neutrophil hypersegmentation?

Answer 8

Mean of 4 lobes. (CAP says 6 or more lobes is the def)
Hypersegmentation of the nucleus.
(Seen in megablastic anemias).

Question 9

What morphology is associated with Alder-Reilly Anomaly?

Answer 9

Alder-Reilly Anomaly
- Deep purple granules in neutrophils.

Question 10

What morphology do you find with Chediak Higashi Anomaly?

Answer 10

Chediak Higashi Anomaly:

Large abnormal cytoplasmic granules in granulocytes, monocytes, and lymphocytes.
(Can be found in all leukocytes types and precursors).