Symptoms and treatment of leukopenia in adults
Author Ольга Кияница
Leukopenia is a decrease in the number of leukocytes in the blood, which is most often a symptomatic condition and accompanies various diseases. The favorable course of the disease is determined by the temporary decrease in white blood cells, the more severe the development of pathology - with chronic decrease.
Leukocytes, or white blood cells, perform a protective function in the body and normally their number is from 4.0 to 10.0 x 10 9 per 1 l of blood.
To determine the number of leukocytes in the first place do a complete blood count, and if necessary, use other diagnostic methods. It is important to recognize the symptoms of leukopenia in time, which are often vague, which allows for proper treatment.
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Leukopenia - what is it in adults?
The term “leukopenia” is a general term referring to a reduced number of leukocytes in peripheral blood. The development of the disease is almost always associated with a decrease in one subgroup of white blood cells. In particular, the following types of leukopenia are distinguished:
- Granulocytopenia is a generic term that refers to all granulocytes, including neutrophils, monocytes, eosinophils, and basophils.
- Neutropenia - the definition indicates a decrease in the number of neutrophils.
- Lymphopenia is a decrease in the number of lymphocytes.
Since monocytes, eosinophils, and basophils make up a relatively small proportion of the total pool of circulating leukocytes, leukopenia is almost always caused by neutropenia or lymphopenia. Therefore, the terms granulocytopenia and neutropenia are often interchangeable. The complete absence of one of the other granulocyte rows is also abnormal and may affect innate immunity, but rarely causes a decrease in the total number of leukocytes in the blood.
The definition of “leukopenia” varies, but in most laboratories the lower limit of the normal total leukocyte count is from 3000 / μl to 4000 / μl. Neutropenia is defined as the absolute number of neutrophils (AKN) less than 1500 / μl.
These indicators were determined mainly in the group of adult Caucasians, and there are several ethnic groups in which the range of the normal number of neutrophils is shifted towards a smaller number. Similar is noted as ethnic neutropenia.
Other features of leukopenia
- Agranulocytosis, the complete absence of granulocytes, belongs to a specific subgroup of neutropenia, which has a unique differential diagnosis.
- A mild or chronic decrease in leukocytes can be benign and, in the absence of alarming symptoms, most often no further diagnosis is made.
- Primary or severe leukopenia, especially neutropenia, which is accompanied by any of the symptoms described below, causes the physician to conduct a thorough diagnosis.
Leukopenia in an adult: causes
There are certain features of the development of the disease, depending on the specific causes and suggesting in the future the most appropriate treatment:
- The age of the patient . Severe congenital neutropenia syndromes are usually present in infancy. Leukopenia is associated with congenital immunodeficiency syndromes determined in childhood.
- Acute currents . Neutropenia, which has been present since childhood, may indicate the presence of congenital syndrome. Leukopenia, which develops acutely, is often caused by drugs with the development of agranulocytosis, acute infections or acute leukemia. Leukopenia, which develops over a period of weeks or months, may be associated with chronic infection or a primary disease of the bone marrow.
- The severity of cytopenia . Although the severity of cytopenia does not help determine its cause, patients with leukocyte counts of less than 800 / μl should be considered immunodeficient. In such cases, appropriate precautions are taken. In the presence of leukocytes of less than 500 / μl against the background of fever, hospitalization measures should be taken for intravenous administration of antibiotics and rapid assessment.
The main reasons for the development of leukopenia in adults, which in these cases is defined as acquired:
- Granulomatous disease
- Autoimmune disease
- Radiation exposure
- Toxic substances
- Malnutrition or improper nutrition
- Protein enteropathy
The diseases that most often provoke the development of leukopenia are hypersplenism, chronic idiopathic neutropenia in adults, rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis.
Pathophysiology of leukopenia
The pathophysiological development of acquired neutropenia, which is most often defined in adults, will be considered.
- Drug agranulocytosis is a rare specific reaction that causes immune destruction of neutrophil precursors in the bone marrow. This is due to the cessation of the action of the excitatory factor, but may be associated with significant morbidity and mortality from sepsis. Many other drugs cause suppression of neutrophil proliferation, depending on the dose. The course of the disease is more benign and can often be tolerated without stopping the drug.
- Autoimmune neutropenia:
- Primary autoimmune neutropenia is caused almost exclusively by antibodies directed against neutrophils, including human neutrophil antigen (HNA1) and CD11b (HNA-4a), two surface antigens or FcγRIIIb, an immune complex receptor involved in the secretion of toxic products. The combination of these autoantibodies leads to the destruction of neutrophils in the spleen or to complement-mediated lysis. This is almost always observed in infants and toddlers. It takes place spontaneously in more than 90% of cases within 1-2 years.
- Secondary autoimmune neutropenia is usually associated with another autoimmune disease, such as Graves disease, Wegener's granulomatosis, rheumatoid arthritis, or systemic lupus erythematosus. Pathogenesis is not clearly defined. Many patients have antibodies against neutrophils, but the relationship between the presence of antibodies and the degree of neutropenia is not well understood.
- Felty's syndrome and large granular lymphocyte syndrome occur in rheumatoid arthritis. Since more than 90% of patients in both groups tested positive for HLA-DR4, it is assumed that these two syndromes reflect the spectrum of a single disease.
- Hypersplenism usually causes only mild neutropenia, often in conditions of mild pancytopenia. Inhibition suppression reflects the increased marginalization of blood cells in the enlarged spleen.
- Lack of nutrients , especially vitamin B12, folate and copper, can lead to neutropenia.
Additionally, it should be noted that chronic idiopathic neutropenia in adults (CINA) is a benign disease, the etiology of which is completely unknown.
Leukopenia - what it is in adults, symptoms
Leukopenia itself is usually asymptomatic. Concomitant constitutional symptoms, including fever, chills, sweating, or weight loss, may indicate the presence of an infection, which may be a cause or consequence of leukopenia. Also, such symptoms are often identified in a malignant neoplasm or autoimmune disorder.
Other signs or symptoms most commonly identified with leukopenia:
- Local signs or symptoms of infection
- Lymphadenopathy (changes in the consistency and size of the lymph nodes)
- Hepatomegaly or splenomegaly (enlargement of the liver or spleen)
- Signs of anemia (pallor, cyanosis, weakness)
- Signs of thrombocytopenia (bleeding on mucous membranes, petechiae, purpura)
- Inflammation of the joints
- Rash of varying severity and localization
Video: Leukopenia (Medical Symptom)
Diagnosis of leukopenia
The following studies were shown to all patients with low white blood cell counts:
- A complete blood count (PAA) is repeated, which allows to confirm the low level of white blood cells and estimate the number of red blood cells and platelets.
- Manual differentiation of blood cells, which helps determine which cell group is reduced, can also be determined by abnormal cells.
- Assessment of previous PAK to determine the severity of leukopenia.
Additionally, those diagnostic methods can be used that provide additional information on the patient's condition. In particular, carried out:
- Complete metabolic profile, including liver enzymes
- Prothrombin time (PT) and partial thromboplastin time (PTT)
- Blood culture
- Human immunodeficiency virus (HIV) test, including serology and viral load
- Polymerase chain reaction (PCR) to determine the viral load of parvovirus, Epstein-Barr virus, cytomegalovirus, herpes simplex virus and hepatitis viruses
- PCR for tick-borne diseases, including rickettsia and anaplasma
- Serum tests for drugs associated with neutropenia or lymphopenia
- Serology of an autoimmune disease (antinuclear antibody, rheumatoid factor)
- Tsifometriya peripheral blood flow markers for B-and T-lymphomas
- Peripheral blood flow cytometry for large granular lymphocytes
- Taking a bone marrow sample followed by a biopsy.
The appearance of a smear from peripheral blood
A smear is important in determining which group of white blood cells is responsible for reducing the total number of leukocytes. In addition, the presence of abnormal forms should be checked. The presence of immature (“left shift”) white cells may indicate an infection or a malignant tumor, especially myelodysplasia or acute leukemia. At the same time, abnormal red blood cells may indicate the presence of autoimmune destruction, myelodysplasia, or bone marrow insufficiency syndrome.
Treatment of leukopenia in adults
Any patient with fever and ACN less than 500 / μl or a constantly decreasing rate of less than 1000 / μl should be hospitalized for early examination and intravenous administration of antibiotics.
The exact choice of antibiotic mainly depends on the degree of resistance and hospital forms. Nevertheless, the following types of drugs are used for antibiotic therapy:
- Third or fourth generation cephalosporins with pseudomonal coating (ceftazidime, cefepime, cefoperazone)
- Anti-pseudomonad penicillins (piperacillin or ticarcillin)
- Carbapenems (imipenem or meropenem)
- Aminoglycosides (gentamicin, tobramycin or amikacin)
- Aztreonam plus fluoroquinolone (levofloxacin or ciprofloxacin)
- Fluoroquinolones should not be used as monotherapy, as resistance can develop quickly.
The use of granulocyte colony-stimulating factor (G-CSF) is controversial. In patients with life-threatening infections and deep neutropenia, G-CSF can shorten the duration of neutropenia. Also, the drug is recommended in cases of overt drug neutropenia. However, since the introduction of G-CSF may complicate the diagnosis of the underlying cause of neutropenia, usually bone marrow sampling and biopsy are performed before starting the introduction of G-CSF. Also, long-acting G-CSF (pegylated G-CSF, non-elastic) is not recommended for acute therapy of febrile neutropenia.
Other treatments that are helpful in reducing complications.
As noted earlier, G-CSF can help reduce the severity of neutropenia of various etiologies. It is assumed that cytokines can stimulate the growth of the main hematological malignancy. Despite the fact that these data are contradictory, the risks are often considered and then the corresponding diagnostic assessment is carried out before the use of G-CSF.Therapy must be carried out under the guidance of a hematologist.
What is the prognosis for leukopenia in adults?
Given the wide range of potential conditions that can lead to leukopenia, the prognosis cannot be determined until the root cause has been established.
Video: What is Leukopenia?