What is hypersplenism

In the diagnosis, doctors can mention splenomegaly( enlarged spleen) and hypersplenism( increasing its function).The spleen is located under the left dome of the diaphragm. In an adult healthy person, it has a length of 16 cm in length, weighing up to 150 g. This is one of the most important organs of the immune system.

In hypersplenism in the spleen, excessive destruction of blood elements occurs, which is manifested by a reduced number of erythrocytes, white blood cells or platelets in the analysis. Hyperplantism is often accompanied by splenomegaly, but the combination is not necessary. An increase in function may occur without splenomegaly.

What are the functions of the spleen?

The main task of the spleen is to fight against foreign agents in the body by producing enough antibodies for the immune system. They allow you to remove from the bloodstream abnormal cells, microorganisms.

The body is covered with a dense capsule. Inside the parenchyma is divided into two layers: red pulp - the main mass, provides a place for the maturation of blood cells, the utilization of foreign particles, white - synthesizes lymphocytes.

In the boundary zone between the layers, the detection and removal of foreign microorganisms occurs

The increase in the spleen is caused by infectious diseases and under normal functional load. The heavy stage of splenomegaly is considered to be its growth of more than 20 cm, weight from 1000 g. The spleen performs 3 main functions:

• development of quality lymphocytes, their maturation and differentiation;
• synthesis of antibodies to foreign agents entering the bloodstream;
• destruction of aged blood cells, their utilization.

In addition, it is involved in the formation of bile, iron synthesis, metabolic processes. Hyperplenism with organ hypertrophy can be primary( its cause is unclear) and secondary, caused by certain diseases.

Primary hypersplenism is divided into congenital( with hemolytic microspherocytic anemia, large thalassemia, hemoglobinopathy), acquired( with thrombocytopenic purpura, neutropenia, pancytopenia).

Secondary hypersplenism causes the following diseases:

• typhoid fever;
• tuberculosis;
• sarcoidosis;
• malaria;
• cirrhosis;
• thrombosis of the portal and splenic vein;
• reticulosis;
• amyloidosis;
• lymphogranulomatosis and others.

How is splenomegaly and hypersplenism revealed?

Large sizes of the spleen detect palpation with the correct position of the patient( lying on the right side).Rarely in specialized clinics, organ scanning with labeled isotopes is used. The increased function of the spleen is indicated by a decrease in the number of blood cells in the usual analysis. Sometimes the indices are compensated by an increase in the hematopoietic activity of the bone marrow.

More accurate data are obtained with the help of ultrasound, computed tomography

. Its load in pathological conditions can increase by 10 times. To exclude the influence of a smear from the bone marrow. Particular attention is paid to the number of reticulocytes, the ratio of the formed elements of red and white blood.

Some diseases are diagnosed by the presence of abnormal( spherical) forms in the peripheral blood of the erythrocytes, target cells( with thalassemia).They are functionally weaker than normal cells, which is confirmed by the definition of osmotic resistance.

When detecting hemolytic anemia, an increased level of indirect bilirubin, an increase in the value of strobobilin in feces analysis, is important. The features of clinical signs of hypersplenism and its treatment we consider on the example of known diseases.

Pathologies accompanied by enlarged spleen

Congenital hemolytic jaundice. Another name for the disease is hemolytic microspherocytic anemia. In 20% of cases, the cause is unknown, the rest is hereditary. Violations cause a defect in the structure of the erythrocyte membrane( membrane).It turns into a good permeable for sodium. This leads to an increase in cell volume and spherical shape.

In the center of the spherocytes there is no clearing

. Erythrocytes become brittle, incomplete, are destroyed in the spleen( hemolysis).Hyperfunction is accompanied by splenomegaly. The disease manifests itself in early childhood. Flows with haemolytic crises. Symptoms are caused by a rapid increase in anemia, jaundice.

Patients appear:

• nausea and vomiting;
• pains at the top of the abdomen;
• heart palpitations;
• shortness of breath;
• the temperature rises;
• the skin becomes at first pale, then icteric.

If the course of the disease is bleeding, the main symptomatology is signs of anemia and mild hemolytic jaundice. It appears later in adolescents and adults. An increased level of bilirubin in half the cases leads to the development of cholelithiasis with attacks of hepatic colic, chronic cholecystitis.

The examination reveals an enlarged spleen, in the blood test microspherocytosis with reduced osmotic resistance of red blood cells, reticulocytosis. On the bone marrow smear, hyperplasia of the erythrocyte sprout is confirmed( in case of crisis - reticulocytopenia and hypoplasia of the red sprout).In the blood, the content of indirect bilirubin increases, in urine urobilin, in feces - stercobilin.

The main effective method of treatment is operative removal of the spleen( splenectomy) in adults during remission, in children aged 3-4 years. As a result, the shape of erythrocytes changes, their life span and stability, anemia and jaundice are eliminated. When a stone is found in the gallbladder, a cholecystectomy is performed.

Large thalassemia

Other names of the disease are Mediterranean anemia, Cooley. Also inherited pathology. The essence of the inferiority of erythrocytes is a violation of protein bonds in hemoglobin. Mild cases are asymptomatic. In severe conditions, patients are prone to any infections.

The examination reveals an enlarged liver and spleen. Typical target cells are found in the blood, the osmotic resistance of erythrocytes is increased. The growth of the number of leukocytes and reticulocytes against a background of a normal platelet level is characteristic.

The serum iron content has been significantly increased, bilirubin concentrations increase moderately. Stones in the gallbladder are found in ¼ patients. Replacement blood transfusions are used in therapy. Splenectomy allows only to get rid of a large organ, but it is not a rational intervention.

In a child with thalassemia, a large head, pronounced jaws, sunken root of the nose are found on the background of yellow skin color.

Thrombocytopenic purpura

Pathology is known as Werlhof's disease, idiopathic thrombocytopenic purpura. A typically persistent low platelet count. Characteristic features:

• temperature increase;
• anemia of hemolytic origin;
• neurological disorders due to cerebral hemorrhages;
• development of renal failure after hemorrhages in the renal parenchyma and proliferation of fibrous tissue.

The spread of infection with the immunodeficiency virus led to a predominance among patients of young men, homosexual orientation, drug addicts. People of both sexes often fall ill, requiring frequent blood transfusions.

In thrombocytopenia, the spleen destroys platelets by a synthesized antiplatelet factor. It is an antibody to the immunoglobulin type G( IgG), less often to IgM, IgA.In addition, there is increased fragility of capillaries, neuroendocrine disorders. The role of bacterial and viral infection is not ruled out.

The causes of thrombocytopenia are composed of increased destruction of platelets by antibodies and a reduction in their lifespan( destruction in the spleen and liver).The disease occurs in acute( in children) and chronic form.

Symptoms:

• hemorrhages on the skin of the chest, extremities, abdomen and in the submucosa( in the mouth, on the eyeballs);
• in women with uterine bleeding;
• nasal and gastrointestinal bleeding, from gum tissue, from minor scratches, injuries;
• blood in the urine;
• signs of hemorrhage in the brain( paresis, paralysis).

Positive symptom of the harness. Spleen only increases in 2% of patients. In the analysis of blood, thrombocytopenia, decreased coagulability, prolonged bleeding time, and absence of retraction of the blood clot are found. In smears from the bone marrow there is a sharp decrease in the number of megakaryocytes of thrombocyte precursor progeny.

Methods of treatment depend on the patient's age, the form and duration of the disease, the response to previous therapy. Apply high doses of corticosteroids, transfusion of platelet mass, splenectomy. The most risky step is the use of surgery in cases where it is impossible to stop bleeding.

Thrombocytopenic changes on the skin have a different form

After splenectomy, they continue therapy with corticosteroids, immunosuppressants( Azathioprine), cytostatic drugs( cyclophosphamide).Complex treatment allows achieving a stable remission.

Felty Syndrome

The disease is rare. It is characterized by a triad of signs: joint damage in the form of rheumatoid arthritis, leukopenia in the blood, splenomegaly( caused by proliferation of red pulp).

It is believed that the disease develops under the influence of a prolonged course of rheumatoid arthritis. In most cases, the patient's spleen begins to produce antibodies to the granulocytes, which leads to their destruction. In the blood, moderate signs of anemia and thrombocytopenia are revealed.

Patients show a tendency to repeat infectious processes( more often sore throats).Exacerbation occurs after another infectious disease. In patients, the temperature rises, agranulocytosis increases in the blood.

The most effective treatment is considered to be a timely splenectomy. A significant increase in the number of leukocytes in the blood test is observed on day 2-3 after the operation. In the therapy of rheumatoid polyarthritis, conventional schemes are used.

Lymphogranulomatosis

Another name is Hodgkin's disease. Pathology refers to the systemic tumor lesion of lymphoid tissue, which includes the parenchyma of the spleen. The specific cause of the disease is unknown. It has been established that men aged 20-30 years from one family are more often affected, the further "peak" falls on the elderly.

According to the frequency of localization:

• in 1 place - cervical lymph nodes;
• on the second - axillary;
• on the third - inguinal.

The spleen, bone marrow, liver, retroperitoneal and lymph nodes of the mediastinum are involved in the process. The histological picture shows a characteristic granulomatous tissue with neutrophils, eosinophils, plasma cells, specific giant Reed-Berezovsky-Sternberg cells.

In rare cases, the initial symptoms are: an increase in paraaortic lymph nodes, a rise in temperature, heavy sweats at night, weight loss. Splenomegaly develops in 40% of cases without hypersplenism. With a significant increase there are dull pain in the left hypochondrium.

The tumor can be localized only in the spleen, it is possible perisplenit( inflammation of the tissues around the capsule), repeated infarctions with severe pain. But this clinical picture is typical for pronounced splenomegaly.

The disease begins with a local enlargement of the cervical lymph nodes, they are soft to the touch, painless and mobile

In the lung tissue, focal or diffuse infiltration occurs, fluid accumulates in the pleural cavity. Lymphogranulomatosis often affects the bone system( spine, pelvis and ribs).The patient has local pain. The involvement of bone marrow is indicated by anemia, leukopenia, thrombocytopenia.

Later, hepatomegaly develops with changes in biochemical tests, bilirubin growth, and a decrease in protein. For the stage of unfolded manifestations are characteristic:

• excruciating skin itch;
• wave-like temperature increase;
• compaction and soldering of lymph nodes;
• symptoms of intoxication;
• depletion.

When squeezing the large lymph nodes of neighboring organs, hoarseness, swelling of the hands and neck( upper vena cava syndrome), persistent diarrhea may develop. In the blood there is a moderate anemia, lymphopenia, neutrophilia, rarely eosinophilia and thrombocytopenia, significantly increased ESR.

Diagnostics use X-ray examination of the mediastinum, ultrasound, magnetic resonance and computer tomography of the liver, spleen, lymph nodes of the peritoneum. For differential diagnosis, the main importance is attached to the detection of Berezovsky-Sternberg cells in the material of lymph node biopsy.

Treatment depends on the stage of the process. X-ray therapy is used in combination with chemotherapeutic agents and corticosteroids. Splenectomy is performed with local development of the tumor in the spleen, expressed by hypersplenism, splenomegaly with signs of compression of the abdominal cavity organs.

Gaucher's disease

Disease refers to metabolic disorders caused by fermentopathies. With it, lipid metabolism is broken in the body. Characteristic manifestations of splenomegaly and hypersplenism.

Pathology is considered hereditary. The basis of violations is caused by deficiency and functional inferiority of β-glucocerebrosidase. This enzyme is necessary for the utilization of lipids.

Glucocerebroside accumulates in macrophages of the spleen, lymph nodes, liver, bone and brain. Rarely are the lungs affected. Pediatric and juvenile forms are characterized by the predominant development of damage to the central nervous system.

The child lags behind the peers mentally and physically, has neurological symptoms( pseudobulbar disorders, epileptiform seizures), hepato- and splenomegaly, pronounced cachexia. In children, the disease is most malignant, leading to death from infectious complications. In adult patients, neurologic symptoms are poorly expressed.

Because of a disturbance in the structure of bone tissue, pain, fractures of limbs and the spine are disturbed. Thrombocytopenia promotes bleeding from the nose and gums, massive bruises on the skin and mucous membranes.

The face and hands are painted yellow-brown in connection with the deposition of hemosiderin, the joints are reddened, swollen, thickening of the conjunctiva of the eyes. The spleen is significantly enlarged, it can occupy almost the entire abdominal cavity, the liver - moderately. There is no jaundice, growth of lymph nodes and ascites.

In the analysis of blood: anemia is small, leukopenia, thrombocytopenia. For final confirmation of the diagnosis, bone marrow or spleen is punctured, specific Gaucher cells are determined in the biopsy.

Splenectomy is the only effective way to treat the disease. Sometimes surgeons retain part of the spleen in order to reduce the risk of developing sepsis. The signs of hypersplenism after the operation gradually disappear. Hyperplenism is not always observed in chronic lymphocytic leukemia, myeloleukemia, AIDS, and long-term infectious diseases.

How is hypersplenism manifested in cirrhosis?

Cirrhosis is considered one of the most common causes of hypersplenism. In the clinical course, the symptoms associated with it are isolated in the "hypersplenism syndrome".Replacing the parenchymal tissue of the hepatic lobules with scarring causes irreversible constriction in the portal vein system.

As a consequence - the development of portal hypertension, stagnation in the underlying vessels, including the spleen. The appearance of hypersplenism on the background of cirrhosis of the liver is indicated by the following symptoms:

• feeling of heaviness, dull pain in the left hypochondrium and half of the abdomen, acute pain may indicate the development of a heart attack, thrombosis of splenic vessels;
• sensation of stomach overflow after eating even a small amount of food is caused by the pressure of the enlarged spleen;
• gum bleeding, nasal bleeding, uterine in women, pallor of the skin( anemia);
• frequent acute infectious diseases due to inhibition of immunity.

In response, the spleen swells and enlarges, shunts( additional connections) are formed to discharge blood to other

systems. In addition to the symptoms of hypersplenism, there are signs of liver damage in the form of:

• of skin and mucous membranes;
• general weakness and loss of strength in muscles;
• weight reduction;
• lack of appetite;
• of vascular "stars" on the skin;
• ascites;
• encephalopathy, expressed in altered behavior, insomnia, depression, disturbed consciousness.

The most important laboratory features are increased hepatic transaminases, alkaline phosphatase, bilirubin, immunoglobulins, protein decrease due to albumin, prothrombin, growth of residual nitrogen and urea, cholesterol, triglycerides, acceleration of ESR.

Disturbance of the function of the spleen is manifested in the fall of hemoglobin, the number of all blood cells. With cirrhosis of special treatment, hypersplenism is not performed. There is no need for removal of the spleen, since all disorders are caused by portal hypertension and the underlying disease.

Hypersplenism supports the course of the underlying disease or is an independent lesion. There are enough diagnostic methods to identify the true cause. Treatment requires a combined regimen. Splenectomy does not always solve all the problems.