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