Insufficient neutrophilic granulocytes
Neutropenia or neutrocytopenia refers to the acute drop in the number of neutrophilic granulocytes in the blood. They are the largest group of white blood cells ( leukocytes ) and are therefore particularly important for the immune system . A lack therefore leads to a severe impairment of the immune system. This is caused by blood formation disorders or increased consumption . The therapy depends on the cause of the disease.
Neutropenia – The most important things at a glance!
- Neutrophilic granulocytes belong to the white blood cells and form the largest group of granulocytes. Therefore, they are important for the immune system.
- They can be detected in the differential blood count. A reduction is referred to as neutropenia or neutrocytopenia, an increase as neutrophilia. Extreme cases are agranularcytosis and granularcytosis.
- The fewer neutrophils in the peripheral blood, the greater the risk of bacterial, fungal and viral infection.
- Neutropenia is caused by impairment of the blood-forming cells in the red bone marrow. These include hereditary diseases, infections, cancer therapy and some medications. It also occurs with increased consumption of leukocytes as a result of allergies and inflammation.
Neutropenia is a symptom, not a disease. Therefore, the therapy is based on the causative underlying disease.
Normal values of neutrophilic granulocytes
| cell label | Proportion of cells in the total leukocytes | cells per per µl |
| neutrophilic granulocytes | ||
| – Rod-nuclear (neutrophil) granulocytes | 3 bis 5% | 150–400 |
| – Segmented (neutrophil) granulocytes | 50 bis 70% | 3000–6000 |
| Eosinophilic granulocytes | 3 bis 4% | 50–250 |
| Basophilic granulocytes | 0 bis 2% | 15–50 |
The neutrophilic granulocytes belong to the white blood cells and are formed in the red bone marrow. It gives it away in an immature form. The precursors with rod-nuclear cell nuclei mature very quickly and become segmented neutrophilic granulocytes . With normal maturation, the full blood count shows 150-400 rod nuclei (3-5 percent) and 3,000-58,000 segmented nuclei (50-70 percent).
If many granulocytes are produced, a left shift occurs in the differential blood count. This means that more immature forms can be detected in the blood.
The decrease in neutrophilic granulocytes is referred to as neutrocytopenia or neutropenia . Only a few cells are found in an agranular cytosis . Values that are higher than the reference range are referred to as neutrophilia , and in extreme cases as granular cytosis .
Causes of congenital neutropenia
A distinction is made between congenital (innate) and secondary (acquired) neutropenia . If the cause is not known, it is called idiopathic neutropenia .
Congenital neutropenia is relatively rare and is due to genetic changes. These include standalone neutropenia such as
- Kostmann’s disease (Kostmann syndrome, congenital infantile agranularcytosis) – usually runs in families, rarely occurs sporadically as an isolated case, with granulocyte maturation arrest and resulting severe agranularcytosis already at birth;
- cyclic neutropenia with a typical cycle of 21 days alternating between neutropenic and normal blood counts
and metabolic disorders associated with neutropenia:
- Shwachmann-Diamond Syndrome (SDS), autosomal recessive inherited with impairments of several organ systems, especially the pancreas (pancreas) with fatty stools, growth disorders and skeletal changes)
- von Gierke’s disease (glycogenosis type 1B) with abnormal accumulation of glycogen in the liver and spleen causing organ enlargement, hypoglycemia and kidney damage;
- Barth-Syndrom.
Causes of neutropenia: decreased production
Factors leading to acquired neutropenia are decreased production or increased consumption. The former is the case with
- Underlying diseases that lead to a reduction in neutrophilic granulocytes in the blood
- viral infections
- measles
- chickenpox
- rubella
- HIV/AIDS
- Mononucleosis
- Hepatitis
- viral infections
- Bacterial infections
- Salmonella enterica (Typhus)
- Mycobacterium tuberculosis (Tuberkulose)
- Rickettsia spec. (diverse Rickettsiosen)
- Brucella spec. (diverse Brucellosen)
- Staphylococcus spec ., especially Staphylococcus aureus – also multi-resistant germs (MRSA)
- Cancer treatment that affects the bone marrow
- chemotherapy
- irradiation
- medication
- Antibiotics (especially penicillin and sulfonamides)
- Antiepileptic drugs (benzodiazepines such as bromazepam and clonazepam)
- Barbiturate
- Anticonvulsants
Causes of neutropenia: increased consumption
There is an increased consumption of neutrophilic granulocytes
- autoimmune diseases
- allergies
- lupus erythematosus
- Hashimoto’s thyroiditis
- autoimmune neutropenia, in which specific autoimmune antibodies destroy neutrophils; especially in women with other immune disorders.
- severe inflammation
- Inflammation of the peritoneum (peritonitis)
- pneumonia (pulmonary)
- blood poisoning (sepsis)
- Poisoning by bacterial endotoxins
- Anaphylaxis (anaphylactic shock).
What causes lead to the disease?
Neutrophilic granulocytes are formed in the bone marrow. If there is damage to the marrow, neutropenia results. For example, the phenomenon arises as a result of chemotherapy or prolonged antibiotic therapy. There is also the possibility that toxic chemicals can trigger the disease. If the patients come into contact with poisonous plants or snake venom, the number of special granulocytes is also reduced. In an organ transplant, the immune system is systematically suppressed by special drugs in order to stop the body’s rejection reaction. This also leads to neutropenia.
If there is an acute inflammation in the body, the body’s own defenses consume numerous neutrophilic granulocytes in order to suppress the inflammatory process. If the inflammation persists for a longer period of time, new cell production comes to a halt: the bone marrow takes too long to form the neutrophilic granulocytes, resulting in a deficiency in the body. After the infection has subsided, the new production of the special white blood cells is regulated. However, the newly formed cells are immature because they are progenitor cells. Technically, the process is called a left shift.
Severe disease increases the risk that neutropenia will follow. This health complication also manifests itself in chronic autoimmune diseases .
Symptoms of neutropenia
Depending on the drop in the neutrophil count in the peripheral blood, different degrees of severity are distinguished depending on the absolute neutrophil count (ANZ):
- mild Neutropenia (ANZ 1000-1500/µl),
- moderate neutropenia (ANC 500-1000/µl) and
- severe neutropenia (ANC less than 500/µL).
A transient, short-lasting decrease is called acute neutropenia . Chronic neutropenia is when it lasts longer than three months.
As neutropenia progresses, the risk of infection increases. Patients with a severe course of the disease should therefore be treated as inpatients.
Skin and mucous membranes that are in direct contact with potential pathogens are particularly affected. If the immune system is weakened, normally harmless roommates have a chance of invading the body. Staphylococci, streptococci and/or fungi primarily affect the respiratory tract and lead to life-threatening infections.
The consequences are
- Fever
- Inflammation of the tonsils (tonsillitis)
- Bronchitis
- Inflammation of the sinuses (sinusitis)
- Middle ear infection (otitis media)
- Inflammation and ulceration of the mucous membranes, such as the oral mucosa (aphthous stomatitis) and the gums (gingivitis)
- nail bed inflammation (paronychia)
- Connective tissue inflammation (phlegmon)
- Blood poisoning (septicemia, sepsis)
- inflammation of the lungs (pneumonia)
The risk of a severe course is particularly high in the case of agranular cytosis. Infections with fungi ( Candida spec .) and viruses also occur more frequently here.
How is neutropenia diagnosed?
If there is an acute inflammation in the body, the body’s defenses use up numerous neutrophilic granulocytes. They take on the task of suppressing the inflammatory process. If the complication persists for a longer period of time, new cell production stops. The bone marrow takes too long to form the neutrophils, resulting in a deficiency in the body. After the infection has subsided, the new production of the special white blood cells is regulated.
The diagnosis takes place in two stages:
- Determination of neutropenia in the blood count
- Clarification of the cause:
- bone marrow
- Inflammation acute/chronic
- Allergic causes
- autoimmune reactions (antibodies)
Treatment of Neutropenia
A drop in white blood cell counts in the differential blood count is a symptom , not a disease. Accordingly, the cause of the disease must be treated. Regularly collected differential blood counts provide information about the progress of the healing process.
If the doctors diagnose a reduction in neutrophilic granulocytes, hygiene is the top priority for those affected. For example, patients frequently wash their hands and use disinfectants. In a severe form of the disease, they use a mouthguard. The purpose is to prevent the ingress of bacteria and viruses. If drug-induced neutrophilia occurs after an organ transplant, the patient needs a single room. The isolation prevents, for example, the infection of other pathogens.
It is also worth stimulating the maturation of the newly formed granulocytes. A specific growth factor, the G-CSF, is used for this.
Drugs with G-CSF are used specifically for treatment. They ensure that neutropenia does not develop. They ensure that the used granulocytes are reproduced more quickly. Alternatively, it has proven useful to begin intensive antibiotic therapy.
The drugs lead to a significant shortening of the neutropenic phase. Both measures can be used alongside chemotherapy to reduce the risk of immunodeficiency and infection.
If allergies and autoimmune reactions are the cause, other therapies are possible, which cannot be briefly presented here.
Leukopenia Therapy
It is good practice to avoid eating undercooked meat. Eat fresh fruit or vegetables, wash the products carefully. At the same time, there is a need to consume enough liquid.
Even with a harmless infection, the reduction in granulocytes leads to fever. Therefore, you should always have the thermometer at hand to carry out a control measurement. If you have a low concentration of white blood cells, it is better to stay away from sick people. Cleaning cloths are also suitable for cleaning the handles of doors or shopping trolleys, for example. In this way you prevent a subsequent infection.
It is also a good idea to avoid eating uncooked meat. Eat fresh fruit or vegetables, wash the products carefully. At the same time, there is a need to consume enough liquid.
Sources:
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