Neutropenia symptoms can go unnoticed, but when you know about its causes and all of its types, it would be easier for you to recognize them on time!
Neutropenia is a pathology that occurs with a decrease in circulating neutrophils in the bloodstream. This condition favors the appearance of secondary infections.
Neutropenia, is the acute or chronic decrease in blood granulocytes, agranulocytosis, especially in the circulating neutrophil count. If this condition is severe, it can put the patient’s life at risk, as he becomes more vulnerable to bacterial and fungal infections.
This pathology is rare, but dangerous, since it has a fatal outcome in 30% of cases. In the United States, very different prevalences have been recorded between ethnic groups, with epidemiological rates of 0.38% in Mexican-Americans, 0.79% in whites, and 4.5% in African-Americans.
It is estimated that there is a 17% chance that it will appear in the chemotherapeutic setting when trying to eradicate a solid tumor. If you want to know everything about this pathology, keep reading.
What is neutropenia?
Neutrophils or polymorphonuclear cells (PMNs) are the most represented immune cells in the circulation, with a concentration of 60-70% of total blood leukocytes.
They are, in diameter, about 10 micrometers and play an essential role in the defense of the body, because they ingest, kill and digest potentially pathogenic microorganisms. These immune cell bodies can do the following:
- Attach to endothelial cells, which line the inside of blood vessels, especially capillaries.
- Migrate to areas where inflammatory responses have occurred. When this cannot be done for whatever reason, it is designated on a cellular basis as abnormal chemotaxis.
- Ingest and destroy pathogenic bacteria for the body.
- Produce antifungal and antimicrobial agents that kill fungi and other germs.
- Forming phagolysosomes, an essential structure for the breakdown and destruction of pathogens.
When these cell bodies are not concentrated enough, an immunodeficiency appears in the patient, which can be mild, moderate or severe. The normal count is 1.5 x 10 ^ 9 / L. From here on, every picture is considered pathological, but with certain differences. According to Health Mapfre professionals, these are the following:
- Mild neutropenia: 1000 to 1500 neutrophils per cubic millimeter of blood.
- Moderate: between 500 to 1000 neutrophils per cubic millimeter of blood.
- Severe: less than 500 neutrophils per cubic millimeter of blood.
The circulating neutrophil count can vary significantly between each patient. For instance, emotional state and time of day can report different data, but always above 1500-1800 cells per cubic millimeter.
At this point, it should be noted that neutropenia does not generate symptoms by itself, since it is the derived infections that cause the characteristic symptomatic picture. Depending on the duration and severity of the disease, the prognosis can range from positive to very reserved. Sometimes a neutropenia can last for months to years.
Neutropenia symptoms and types
Neutropenia can be severe or acute, as indicated in the MSD Manual:
According to a publication in Fcarreras, an acute neutropenia is one that lasts less than 3 months. Its physiological bases are the use or rapid destruction of neutrophils or, failing that, impaired production.
The initial limit of chronic neutropenia is usually established when it exceeds 3 months in duration. It can last for years, and finds the physical cause in reduced production or excessive splenic sequestration. The latter variant manifests with an abnormal enlargement of the spleen.
Causes of neutropenia
In this section we have a lot to cut, since neutropenia has a multifactorial etiology. Scientific research «Neutropenia: causes and consequences» helps us to elucidate the causes along the following lines.
Intrinsic disorders in the maturation and proliferation of blood cells
There are many rare pathologies that can disrupt myelopoiesis. But severe congenital neutropenia (SCN) is the worst, which consists of an arrest in the maturation of cells that are going to become neutrophils.
Symptoms begin from infancy, as newborns have concentrations below 200 / mcL. According to an article in the Annals of Pediatrics, 79% of infants with this condition get diagnosis before the first year of life. They require administration of antibiotics usually.
This condition can be dominant or recessive, X-linked, or sporadic. Several genes related to it have been isolated.
This is a very rare cause of neutropenia. It is of exceptional appearance and it is an autosomal dominant disorder, that is, linked to a non-sexual chromosome and which occurs independently of the allele with which the mutated sequence is paired.
Glycogenosis type 1
Type I glycogen storage disease (GSD), characterized by low tolerance to fasting, growth retardation, and hepatomegaly (inflammation of the liver), is associated with the occurrence of repeated neutropenic infections.
The same mechanisms that promote the accumulation of glycogen and fat in the liver prevent the proper transport of neutrophils. The bone marrow shows a hypercellular state, as neutrophils accumulate in it, but the amount of circulating cellular elements in the blood is lower than normal.
As indicated by the Mayo Clinic, in aplastic anemia the bone marrow has an absent (aplastic) or very low (hypoplastic) cell concentration. This is because hematopoietic stem cells, precursors of all circulating blood elements, have a damage and cannot give rise to the different cell lines.
Bone marrow replacement and other treatments (chemotherapy)
The invasion of abnormal cells in the bone marrow results in neutropenia, since this tissue is responsible for producing neutrophils. Some cancer treatments, such as chemotherapy, can also destroy white blood cells and other circulating cell bodies.
There are many other causative agents of neutropenia, such as chronic folic acid deficiency, certain serious infectious events, the use of certain medications, and others.
As we have said previously, neutropenia does not cause symptoms by itself, but rather facilitates secondary infections, which do manifest with specific clinical signs. In general, the symptom that elucidates a neutropenia is that the patient will have infections more frequently, these will be more serious and will get worse in a short time.
The effects of the associated pictures usually present clinical signs such as the following:
- Development of ulcers or pustules.
- Swollen lymph nodes.
- Among others.
These depend on the etiological agent in the body by the neutrophil deficiency, not on the condition itself.
Diagnosis of neutropenia
Neutropenia is easy to identify. If a patient suffers from more infections than normal, the doctor will perform a complete blood count (blood test), which will detect the decrease in circulating neutrophils. This is the simple part of the diagnosis, since the complex thing is trying to find the underlying reason.
Depending on the family history, symptoms and clinical pictures, doctors will perform genetic tests, take samples of the bone marrow (biopsy) and do other additional tests. Some of them are the serum immunoglobulin count, the phagocytic function test or the complement system evaluation.
Human granulocyte colony stimulating factor (G-CSF) can increase the number and functionality of circulating neutrophils. The prophylactic use of antibiotics and antifungals, meanwhile, will prevent the patient from suffering the most common infections in immunosuppressed people.
Some newer treatments, such as gamma interferon, have shown their usefulness in preliminary studies in patients with this disease. Bone marrow transplantation can save the most seriously ill, but there is a significant probability of rejection, which carries a not inconsiderable mortality rate.
Each treatment option depends on the underlying cause of the condition.
What to remember about neutropenia?
As you can see, neutropenia is a single clinical event (reduction in circulating neutrophils), but it can have many causes. From chemotherapy to genetic diseases, various conditions can prevent the development, release, or functionality of neutrophils.
If the condition is due to an infection, you will return to normal once you treat it. If, on the other hand, it is a chronic condition, you must treat it long-term. You will do that with antibiotics and therapies that stimulate the production of neutrophils.
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