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Differentiating congenital pelger-huet anomaly and acquired pseudo-pelger huet anomaly- 5 things to consider.

Pelger huet anomaly is a benign condition which is inherited in an autosomal dominant fashion. It is caused due to mutations in Lamin B receptor ( LBR)

On the flip side, pseudo-pelger huet is described in conditions where neutrophils in the peripheral smear resemble pelger-huet cells but are not associated with the familal pelger-huet anomaly.

CONDITIONS ASSOCIATED WITH THE PRESENCE OF PSEUDO-PELGER HUET CELLS

1. Myelodysplastic syndrome

2. Acute myeloid leukemia

3. Chronic myelogenous leukemia

4. Infections and inflammatory conditions

It is important to differentiate between congenital pelger-huet anomaly and acquired pseudo-pelger huet anomaly to avoid unnecessary testing and treatment.

Congenital pelger-huet anomaly is a benign condition and needs no treatment, on the other hand acquired pseudo-pelger huet anomaly often has an underlying condition that has to be treated.

Here are 5 things to consider while differentiating congenital pelger-huet anomaly and acquired pseudo-pelger huet anomaly.

1. AGE

Is the patient young or old?

Congenital pelger-huet anomaly- Age of the patient is generally young less than 30 years according to published reports of the condition.

Acquired pseudo-pelger huet anomaly- patients are often older than 60 years if age.

This is an important fact to consider while differentiating the two entities.

2. FAMILY HISTORY

Is there presence of family history or not?

Congenital pelger-huet anomaly is Autosomal dominant and often has family history of similar findings in siblings, children and parents.

No family history is present in acquired pseudo-pelger anomaly.

3. HEMATOLOGICAL MANIFESTATIONS

Congenital pelger-huet anomaly is not associated with any other hematological manifestations.

Acquired pseudo-pelger huet anomaly which can be secondary to myelodysplasia or leulemias may have other hematologic manifestations.

4. SKELETAL ANOMALIES

Congenital pelger-huet anomaly is associated with skeletal anomalies such as post-axial polydactyly, short metacarpals, short upper limbs, short stature, or hyperkyphosis.

These manifestations are absent in pseudo-pelger huet anomaly.

5. MORPHOLOGY OF NEUTROPHILS

In 1955, Ham classified neutrophils based on their morphology into Type A, Type B and Type C.

Ham’s classification- 1995

In acquired pseudo-pelger anomaly- Type A neutrophils predominate.

In congenital heterozygous pelger-huet anomaly- Type B neutrophils predominate.

In congenital homozygous pelger-huet anomaly- Type C neutrophils predominate.

Morphology of neutrophils in pelger-huet anomaly

These are the 5 things to consider when differentiating congenital pelger-huet anomaly and acquired pseudo-pelger huet anomaly.

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