Erythrocytosis : Causes, Symptoms, Diagnosis & Treatment

• Classification
• Why Does Erythrocytosis Occur?
• Physiological Causes
• Respiratory Hypoxia
• Circulatory Hypoxia
• Hemic Hypoxia
• Exogenous Hypoxia
• Kidney Disease
• Disorders Of Neurohumoral Regulation
• Endocrine Pathologies
• Polycythemia
• Hereditary Familial Erythrocytosis
• Fluid Loss
• Diagnostics
• Treatment
• Conservative Therapy
• Surgery

Erythrocytosis is an increase in the level of red blood cells in the blood. May be physiological or pathological. It accompanies diseases of the bronchopulmonary system, heart defects, kidney diseases, hormonally active neoplasms, high blood pressure, significant fluid loss. Manifestations vary, moderate hepatosplenomegaly, red cyanosis of the skin, varicose veins, sometimes bleeding, thrombosis are possible. Erythrocytosis is determined by the results of a clinical blood test. Treatment includes therapy of the underlying pathology, lifestyle correction, diet, bloodletting.

Classification

Erythrocytosis is diagnosed when the following indicators are exceeded: in men - 5.5x109 / ml, in women and children under 13 years old - 4.7x109 / ml. In newborns, the number of red blood cells should exceed 5.6x109 / ml. The following types of erythrocytosis are distinguished:

1. By the presence or absence of connection with pathological processes:

• Physiological . The change in the indicator is not associated with diseases. Occurs as part of adaptation to hypoxia due to environmental conditions.
• Pathological . It accompanies some diseases, develops as a result of homeostasis disorders caused by various traumatic and non-traumatic causes.

2. According to the changes underlying erythrocytosis:

• Absolute . It becomes a consequence of increased erythropoiesis against the background of hypoxia, increased production of erythropoietin due to kidney disease, endocrine diseases, hormonally active tumors.
• Relative . Formed after a decrease in plasma volume as a result of profuse sweating, vomiting, diarrhea.

3. By the time of occurrence:

• Primary . It is hereditary, provoked by gene mutations that cause a change in the structure of hemoglobin or an enzyme that is responsible for transporting oxygen to red blood cells, its subsequent “return” to tissues.
• Secondary (symptomatic) . It develops throughout life, potentiated by diseases and pathological conditions.

Symptomatic erythrocytosis is the most common type of this condition. It is detected in men and women, it can be absolute or relative. Absolute symptomatic erythrocytosis is detected more often than relative, provoked by the following factors:

• Kidney diseases : ischemia of various etiologies, neoplasms, condition after kidney transplantation.
• Disturbances of neurohumoral regulation : excessive stimulation of the autonomic nervous system.
• Endocrine disorders : increased levels of thyroid hormones, glucocorticoids, catecholamines, adrenocorticotropic hormone.
• Blood diseases : hemoblastosis with increased production of red blood cells.
• Hemic hypoxia : poisoning by substances that reduce the oxygen capacity of the blood.
• Circulatory hypoxia : insufficient blood supply to organs and tissues due to dysfunction of the heart.
• Respiratory hypoxia : a decrease in lung ventilation against the background of bronchopulmonary pathologies.
• Exogenous hypoxia : normobaric hypoxia with a lack of oxygen in the air, hypobaric hypoxia with decompression sickness.

Among the relative symptomatic erythrocytosis, hemoconcentration predominates - arising from a decrease in the amount of fluid in the vascular bed. Less common in women and men are redistributive erythrocytosis - conditions in which there is a sharp release of red blood cells from the depot.

Erythrocytosis

 

Why does erythrocytosis occur?

Physiological causes

A high number of erythrocytes is detected in residents of high mountain areas. Even in the absence of somatic diseases, the body suffers from hypoxia, due to a decrease in the amount of oxygen in the external environment, more blood cells are produced compensatory to transport it. In native residents of the highlands, physiological erythrocytosis is observed throughout life, in visitors it develops during a long stay in the area.

Another example is erythrocytosis in infants after birth. In the womb, the blood of the fetus was saturated with oxygen from the blood of the mother. There is less oxygen in a woman's blood than in air, so an unborn child needed a lot of red blood cells to carry it. With the beginning of breathing with atmospheric air, the supply of oxygen increases, the number of blood cells gradually decreases.

Respiratory hypoxia

Erythrocytosis is detected in pathologies accompanied by a decrease in the volume of pulmonary ventilation:

• Bronchial asthma. It can be allergic, non-allergic (for example, aspirin), mixed. An attack of suffocation is accompanied by a feeling of tightness in the chest, a reduction in the duration of inspiration, an increase in the duration of exhalation, wheezing, forced position, swelling of the neck veins.
• Chronical bronchitis. It can be obstructive or non-obstructive. Manifested by cough with mucopurulent sputum. The amount of sputum increases during an exacerbation. Symptoms of obstruction are expiratory dyspnea, persistent cough, wheezing, and swelling of the jugular veins.
• COPD Characterized by a progressive course of bronchitis or emphysematous type. In the first case, symptoms of obstructive bronchitis predominate, in the second - expiratory dyspnea.
• Restrictive Violations . Chronic pneumonia, pneumosclerosis, pneumothorax, exudative pleurisy, widespread adhesions in the pleural cavity, and other conditions lead to restriction of lung tissue movements.

Along with nonspecific diseases, various forms of pulmonary tuberculosis and pneumoconiosis play a significant role in the development of respiratory hypoxia with symptomatic erythrocytosis: silicosis, silicosis. In addition, this type of hypoxia is observed in smokers. It is caused by the constant intake of tobacco smoke, the occurrence of smoker's bronchitis (a type of COPD). Men are more often affected.

Circulatory hypoxia

The phenomena of hypoxia are most pronounced with blue heart defects, accompanied by venous discharge or mixing of blood with hypovolemia of the pulmonary circulation, hypoxemia. Secondary erythrocytosis and the development of collaterals to a certain extent allow compensating for hemodynamic disorders. Due to the threat of irreversible dystrophic changes in the myocardium of children, it is recommended to operate at an early age. Erythrocytosis is characterized by the following defects:

• Fallot's disease: triad, tetrad, pentad;
• tricuspid valve atresia;
• transposition of the great vessels;
• anomalies of the confluence of the pulmonary veins;
• common arterial trunk.

Hemic hypoxia

A decrease in the oxygen capacity of the blood and compensatory erythrocytosis are potentiated by some exogenous intoxications. Carbon monoxide poisoning occurs at home or at work. Accompanied by a pressing headache, nausea, dizziness, unsteady gait. Sometimes there are convulsions, coma. Subsequently, retrograde amnesia, cardiac conduction disorders, pneumonia, bronchitis are possible.

Nitrate poisoning is provoked by the use of drinking water, agricultural products grown with the use of nitrogenous fertilizers, the intake of large doses of certain pharmaceuticals, and the inhalation of toxic fumes. Manifested by gastrointestinal disorders, jaundice, shortness of breath, hypotension, impaired consciousness.

Exogenous hypoxia

At normal atmospheric pressure, but limited air flow, normobaric hypoxia occurs. Erythrocytosis is observed in victims of natural disasters, industrial accidents (for example, staying in a confined space without air access after a collapse in a mine). The cause of hypobaric hypoxia is a decrease in barometric pressure during a long stay at high altitude in pilots. An extremely rapid change in environmental conditions with the development of decompression sickness is noted.

kidney disease

Insufficient blood supply to the kidneys, the presence of volumetric formations of the organ stimulates increased secretion of erythropoietin, activation of the red hematopoietic germ. Erythrocytosis is accompanied by:

• Pyelonephritis. Acute inflammation is characterized by fever, weakness, dull pain in the lumbar region, discoloration of urine. In the chronic process, the symptoms are smoothed out, the general condition is not disturbed.
• Glomerulonephritis. The acute form proceeds with hyperthermia, signs of intoxication, hematuria, hypertension, edema, more pronounced on the face. For chronic glomerulonephritis, a relapsing course is typical with the periodic appearance of a clinic of acute inflammation.
• Hydronephrosis. Occurs due to obstruction of the outflow of urine. Manifested by discomfort, dull pain in the lower back, which are further joined by progressive fatigue, transient hypertension, hematuria.
• Stenosis of the renal arteries. Increasing arterial hypertension, ischemic nephropathy are noted. The first syndrome includes cerebral manifestations, the second - back pain, sometimes hematuria. Development of secondary hyperaldosteronism is possible.
• Kidney cyst. Erythrocytosis develops with compression of blood vessels and stimulation of the juxtaglomerular apparatus, accompanied by an increase in blood pressure. Other possible symptoms are pain, urodynamic disturbances.

The list of neoplasms that provoke erythrocytosis includes nephroblastomas, hypernephromas, neoplasias that secrete erythropoietin. Sometimes the rate increases in patients who have undergone kidney transplantation.

Disorders of neurohumoral regulation

Increased activity of the sympathetic nervous system, causing erythrocytosis, accompanies chronic heart failure, neurogenic essential arterial hypertension. A similar change in the level of erythrocytes against the background of neurohumoral disorders is observed in severe sleep apnea or frequent panic attacks.

Endocrine pathologies

Some hormones increase the level of oxygen utilization, so their overproduction is accompanied by secondary erythrocytosis:

• Thyroid hormones . Hyperthyroidism is observed with diffuse toxic goiter, autoimmune thyroiditis, toxic adenoma of the thyroid gland.
• Adrenocorticotropic hormone . Hypercorticism is determined with pituitary microadenoma, Itsenko-Cushing's syndrome caused by adenomas and adenocarcinomas of the adrenal glands, ACTH-ectopic syndrome.
• Catecholamines and glucocorticoids . An elevated level of hormones is found, respectively, in tumors of the medulla and cortical layer of the adrenal glands.

In addition, erythrocytosis is diagnosed in people taking androgenic steroids as a dope to stimulate muscle growth, improve athletic performance.

Polycythemia

Erythremia (polycythemia or Wakez's disease) is a chronic leukemia, accompanied by increased proliferation of all hematopoietic lineages, primarily erythrocyte. Typical signs of the disease are plethora (telangiectasia, red or cherry skin tone, scleral hyperemia), unbearable pruritus. Many patients have erythromelalgia. With progression, bone pain, migraine, hypertension, myalgia are observed.

hereditary familial erythrocytosis

It is a group of genetically determined diseases with a change in the structure of hemoglobin, metabolic disorders in red blood cells, excessive excretion of erythropoietin by the kidneys. The severity and time of onset of symptoms are determined by the form of hereditary familial erythrocytosis. Possible tachycardia, varicose veins, hemorrhoids, hepatosplenomegaly, shortness of breath, increased bleeding.

Fluid Loss

Hemoconcentration with the formation of erythrocytosis occurs with significant fluid loss. In a healthy person, a transient violation of the laboratory indicator can be determined with prolonged thirst, after profuse sweating when working in conditions of high ambient temperature. The latter option is more common in men engaged in heavy physical labor. Erythrocytosis is also found in the following conditions:

• Intestinal infections: cholera, dysentery, salmonellosis. Fluid is lost with repeated vomiting, profuse diarrhea with watery stools.
• Extensive burns: more than 15% of the body surface with burns of II and more severe degrees. The cause of hemoconcentration is the release of burn exudate.
• Ketoacidosis: ketoacidotic coma in diabetic patients. Fluid is excreted from the body through the kidneys along with glucose.

RBC count

 

Diagnostics

Lung diseases are diagnosed by pulmonologists. Patients with suspected kidney pathology are examined by nephrologists. For other diseases, the participation of hematologists, oncologists, and other specialists is required. Erythrocytosis is confirmed by data from a complete blood count. Other indicators determined during the study are the levels of hemoglobin and hematocrit, the number of reticulocytes, leukocytes, and platelets.

Leukocytosis with a shift of the formula to the left indicates the presence of an inflammatory process. Reticulocytosis is detected in bronchial obstructive diseases, restrictive lesions of the lung tissue, cyanotic heart defects, polycythemia. Wakez disease is also characterized by an increase in the level of platelets and leukocytes, microcytosis is possible.

To clarify the diagnosis, the patient is prescribed an extended laboratory examination. Produce determination of the level of erythropoietin, the study of the gas composition of the blood. If endocrine diseases are suspected, hormonally active tumors are analyzed for adrenal hormones, thyroid gland, ACTH. Patients with polycythemia undergo a trephine biopsy followed by a histological examination. The list of instrumental techniques depends on the nature of the pathology:

• Diseases of the lungs . The basic study is a chest x-ray. The pictures show signs of bronchitis, pneumonia, pneumosclerosis, tuberculosis, and other diseases. The method can be supplemented with CT, MRI, bronchoscopy, spirometry, pneumotachography.
• Heart defects . The list of diagnostic procedures includes electrocardiography, phonocardiography, Holter monitoring, echocardiography, chest radiography. Patients with pulmonary hypertension are shown angiography, sounding of the heart cavities.
• Kidney disease . Patients are prescribed ultrasound, MRI, CT of the kidneys. Pyelography, excretory urography, nephroscintigraphy are performed. To identify secondary disorders from other organs, an examination of the fundus, ECG, ultrasound of the pleural cavity is performed. To clarify the nature of the pathological process, a biopsy with a morphological study is performed.
• Endocrine Disorders . Depending on the existing symptoms, ultrasound, CT or MRI of the adrenal glands or thyroid gland, X-ray of the Turkish saddle, contrast MRI of the brain may be recommended.

Treatment

Conservative therapy

Smokers are advised to give up bad habits. Diet and physical activity regimen are selected taking into account the characteristics of the disease. Symptomatic treatment consists in bloodletting. To eliminate erythrocytosis, a course is prescribed, including 3-4 procedures with a simultaneous removal of 400-50 ml of blood.

When determining the need for manipulation, the compensatory role of erythrocytosis in patients with heart defects is taken into account, according to indications, oxygen therapy is used, and antiplatelet agents are used. In renal pathologies, bloodletting is carried out until the hematocrit normalizes. In recent years, the procedure has been performed less frequently than in the past, as studies show bone marrow stimulation and subsequent hematopoiesis after bloodletting.

Etiopathogenetic therapy for hereditary erythrocytosis has not been developed. Antiplatelet agents, anticoagulants, and sometimes cytotoxic agents are recommended as symptomatic measures. Secondary erythrocytosis is eliminated by treating the underlying pathology:

• Kidney diseases . With pyelonephritis, antibacterial and detoxification therapy, immunostimulants, immunomodulators are indicated. Patients with glomerulonephritis are prescribed immunosuppressive therapy using cytostatics, glucocorticoids, NSAIDs, anticoagulants and antiplatelet agents.
• Bronchopulmonary pathologies . Effective bronchodilators, mucolytics. During periods of exacerbation of inflammatory diseases, antibacterial agents are recommended. Patients with COPD and bronchial asthma should use inhalers, nebulizers, spacers. In tuberculosis, anti-tuberculosis drugs are effective.
• Endocrine disorders . In diabetes mellitus, correction of glucose levels is necessary by administering insulin or hypoglycemic agents. Hyperthyroidism requires thyreostatics. Itsenko-Cushing's syndrome involves treatment with drugs that suppress steroidogenesis. Radiation therapy and dopamine agonists are effective in pituitary microadenomas.

Surgery

With secondary erythrocytosis, the following operations can be performed:

• Respiratory hypoxia : drainage of the pleural cavity, thoracostomy, thoracoplasty, pleurectomy, lung resection.
• Circulatory hypoxia : aortic and pulmonary artery transplant, Norwood operation, Fontan operation, heart transplant.
• Kidney pathologies : bougienage, stent placement, lithotripsy and removal of calculi to restore the passage of urine, hydronephrosis plasty, excision of a kidney cyst, removal of a kidney tumor, kidney resection, nephrectomy.
• Endocrine diseases : resection of the thyroid lobe, hemithyroidectomy, removal of the thyroid gland, adrenalectomy, excision of the pituitary tumor.