The Pelger neutrophil anomaly is a fairly rare disease. This pathology is hereditary. It is characterized by congenital changes in the nuclei of neutrophils. How dangerous is such an anomaly in the structure of blood cells? And how does it manifest? We will answer these questions in the article.
Neutrophils: general information
Not every patient knows about such a pathology as the Pelger neutrophil anomaly. What is this disease? To answer this question, let's try to understand the structure and functions of neutrophils.
Neutrophils are the largest variety of white blood cells. They are necessary for the body to deal with pathogens of infectious diseases. These cells rush to the focus of inflammation. and then absorb and digest dangerous bacteria and viruses. This process of neutralizing foreign agents is called phagocytosis.
In the decoding of a blood test, 4 types of neutrophils are usually indicated:
- Myelocytes. These are the youngest cells. Normally, they can only be present in the bone marrow. Their presence in peripheral blood always indicates pathology.
- Young cells. They may be present in a blood test in very small quantities (up to 1% of the total number of neutrophils);
- Band neutrophils. This is a small group of cells. They make up 1 - 5% in the norm.
- Segmented cells. It is this type of neutrophils that predominates in peripheral blood (40 - 70%).
Mature segmented neutrophil species include only segmented cells; all other species are considered young forms.
With the Pelger neutrophil anomaly, changes in the structure of segmented cells are noted. They become like young forms. This often leads to errors in the diagnosis of various pathologies.
Blood picture
This congenital disorder can only be detected using a clinical blood test. If the nuclei of mature cells have few segments and change shape, this may indicate the Pelger neutrophil anomaly. In the photo below you can see pathological changes in the nuclei.
The core of Pelger neutrophils can take the following forms:
- oval;
- rod-shaped;
- horseshoe-shaped;
- bean-shaped;
- rounded.
In more rare cases, there are nuclei with a constriction in the center, similar to a gymnastic weight.
In this case, the core looks too short and consists of only one segment. It has a lumpy structure. Less common are nuclei of a modified form, having two or three segments.
If the nucleus of a segmented neutrophil has a round shape, then it can be very easily mistaken for a myelocyte. Only a detailed blood test can show that this is not a young, but a mature cell. The presence of neutrophils with altered nuclei often leads to errors in the interpretation of the analysis results.
Causes
An anomaly occurs due to a violation of the formation of segments of neutrophilic nuclei. The reason for it is a failure at the genetic level.
This disease is hereditary. It is transmitted in an autosomal dominant manner. This means that pathology can be transmitted to children if both parents are carriers of the defective gene.
Anomaly occurs in 1 child out of 1000 newborns. It is equally common in boys and girls. Pathology is benign. Many doctors consider the Pelger anomaly not a disease, but a constitutional feature.
Manifestations
Any pathological symptoms with Pelger’s neutrophil anomaly are completely absent. This innate feature does not affect the well-being of a person and does not pose a health hazard.
Changing the shape of the neutrophil nuclei does not affect phagocytosis. Such cells retain the ability to fight foreign microorganisms and produce enzymes. On the state of immunity, this anomaly is not reflected in any way.
A very small proportion of doctors believe that patients with Pelger’s neutrophil abnormality are more likely to have low growth and a tendency to bend bones. However, these data are not scientifically confirmed. Most people with this feature have normal growth and do not suffer from bone pathologies.
Effects
What does the Pelger neutrophil anomaly lead to? This violation does not give any complications and negative consequences for human health.
Difficulties arise only when interpreting the results of a blood test. Very often, doctors take altered segmented neutrophils for stab counts. And an increase in the concentration of "sticks" may be a sign of the following diseases:
- oncological diseases of the hematopoietic system;
- bacterial and viral infections;
- injuries and burns;
- myocardial infarction;
- inflammatory processes in different organs;
- allergic reactions;
- sepsis;
- parasitic diseases;
- renal failure.
With an altered blood picture, the doctor may suspect the above pathologies in the patient. In the history of medicine, there have been cases when patients with the Pelger neutrophil anomaly were mistakenly diagnosed with infectious diseases or leukemia. As a result, people underwent a completely unnecessary course of treatment.
This innate feature often leads to an erroneous interpretation of the clinical blood test data. However, a qualified and experienced doctor will never make a diagnosis using only one test.
Diagnostics
If in the clinical analysis the patient has signs of Pelger's abnormality, then the doctor must necessarily indicate this in the transcript. To avoid errors in the diagnosis, a person needs to additionally pass a blood smear for the following indicators:
- kernel maturity;
- chromatin density;
- neutrophil ratio.
With the Pelgerian anomaly in the patient, the chromatin density remains normal, the nucleus has an altered shape, but it is old. This study helps distinguish modified mature cells from young forms.
If possible, then a clinical analysis and a blood smear should be taken from the patient's parents. Since the pathology is hereditary, the same anomaly is noted in the mother and father of the patient. This is an important diagnostic sign.
Do I need treatment
Clinical manifestations of the Pelger neutrophil anomaly are completely absent. A person with this feature is considered absolutely healthy and does not need treatment.
It is advisable to detect an anomaly in childhood. This will help to avoid diagnostic errors in the future. If a patient with a Pelger neutrophil anomaly has to undergo a blood test, then it is very important to tell the doctor about his innate features. Otherwise, the test results will be interpreted incorrectly.
Similar anomalies
We examined the innate form of the Pelger anomaly. However, similar changes in neutrophils may also occur in the following diseases:
- systemic lupus erythematosus;
- tuberculosis
- malaria
- flu
- lymphogranulomatosis;
- decreased thyroid function;
- leukemia.
In these cases, the changes are acquired. They are noted not constantly, but only with an exacerbation of the underlying pathology.
There is also a very rare congenital disease, which geneticists call SOPH syndrome. It was described for the first time in 2009. This pathology is found in the peoples of the North, mainly in the Yakuts. The Pelger anomaly is only one of the manifestations of this syndrome. This genetic disease is accompanied by other pathological signs:
- short stature;
- sagging skin;
- atrophy of the optic nerves and retina.
If the child has a Pelger anomaly, then you should carefully look at his health and state of health. If no other pathological signs are observed, then there is no reason for concern. This constitutional feature does not affect either the duration or quality of life.