Splenomegaly

Splenomegaly is almost always secondary to other diseases, which are very many, as well as possible ways to classify them.

Myeloproliferative and lymphoproliferative diseases, accumulation diseases (eg, Gaucher's disease) and connective tissue diseases are the most common causes of splenomegaly in temperate countries, while infectious diseases (eg malaria, kala-azar) prevail in the tropics.

[1], [2], [3], [4], [5], [6], [7], [8]

Causes of splenomegaly

The cause of pronounced splenomegaly ( spleen is palpated 8 cm below the costal arch) are usually the following diseases: chronic lymphocytic leukemia, non-Hodgkin's lymphoma, chronic myelogenous leukemia, true polycythemia, myelofibrosis with myeloid metaplasia, and hairy cell leukemia.

Splenomegaly can lead to cytopenia.

Congestive splenomegaly (Bunti's disease)

• Cirrhosis.
• External compression or thrombosis of the portal or splenic vein.
• Individual disorders of vascular development

Infectious or inflammatory diseases

• Acute infections (eg, infectious mononucleosis, infectious hepatitis, subacute bacterial endocarditis, psittacosis).
• Chronic infections (eg, miliary tuberculosis, malaria, brucellosis, Indian visceral leishmaniasis, syphilis).
• Sarcoidosis.
• Amyloidosis.
• Connective tissue diseases (eg SLE, Felty syndrome)

Myeloproliferative and lymphoproliferative diseases

• Myelofibrosis with myeloid metaplasia.
• Lymphomas (for example, Hodgkin's lymphoma).
• Leukemia, especially chronic lymphocytic leukemia and chronic myelogenous leukemia.
• True polycythemia.
• Essential thrombocythemia

Chronic, usually congenital, hemolytic anemia

• Anomalies in the shape of the erythrocyte (eg, congenital spherocytosis, congenital elliptocytosis), hemoglobinopathies, including thalassemia, sickle-cell variant of hemoglobin (eg hemoglobin SC disease), congenital hemolytic anemia of Heinz bodies.
• Erythrocyte enzymes (eg, pyruvate kinase deficiency)

Disease of accumulation

• Lipid (eg, diseases of Gaucher, Niemann-Pick, Hend-Schüller-Krischer).
• Non-lipid (for example, the disease Letterter-Sieve).
• Amyloidosis

Spleen cyst

• It is usually caused by the resolution of the previous hematoma

[9], [10], [11], [12], [13],

Symptoms of splenomegaly

Splenomegaly is a criterion of hypersplenism; The size of the spleen correlates with the degree of anemia. You can expect an increase in the size of the spleen by about 2 cm below the costal arch for every 1 g of hemoglobin reduction. Other clinical signs, as a rule, depend on the manifestations of the underlying disease, sometimes there is pain in the spleen. Until other mechanisms exacerbate the manifestations of hypersplenism, anemia and other types of cytopenia are mild and asymptomatic (for example, platelets from 50,000 to 100,000 / μL, the number of white blood cells from 2500 to 4000 / μL with normal leukocyte differentiation). The morphology of erythrocytes is usually normal, with the exception of rare spherocytosis. Reticulocytosis is a common occurrence.

Hyperplenism is suspected in patients with splenomegaly, anemia or cytopenia; Diagnosis is similar to the situation with splenomegaly.

Examination with splenomegaly

[14], [15], [16], [17], [18],

Anamnesis

Most of the detected symptoms are due to the underlying disease. However, splenomegaly itself can cause a sense of rapid saturation as a result of the influence of the enlarged spleen on the stomach. There are feelings of heaviness and pain in the left upper quadrant of the abdomen. The expressed pain in the left side suggests the presence of a spleen infarction. Recurrent infections, symptoms of anemia, or bleeding manifest in the presence of cytopenia and possible hypersplenism.

[19], [20], [21], [22], [23], [24]

Inspection

The sensitivity of the method of palpation and percussion in determining the increase in the size of the spleen is 60-70% and 60-80%, respectively, with the documented ultrasonic splenomegaly. Normally up to 3% of people have a palpable spleen. In addition, the palpable mass in the left upper quadrant may be due to another cause, rather than an enlarged spleen.

Other additional symptoms are spleen friction noise, suggesting the presence of a spleen infarction, and epigastric and splenic sounds that indicate congestive splenomegaly. In generalized adenopathy, it can be assumed that there is a myeloproliferative, lymphoproliferative, infectious or autoimmune disease.

Diagnosis of splenomegaly

If it is necessary to confirm splenomegaly in cases of questionable result after preliminary examination, ultrasound examination is the method of choice due to its high accuracy and low price. CT and MRI can provide a more detailed image of the organ. MRI is especially effective in determining portal thrombosis or thrombosis of the splenic vein. Radioisotope research is a highly accurate diagnostic method that can identify additional details of the splenic tissue, but the method is very expensive and difficult to perform.

Specific causes of splenomegaly, identified during clinical examination, should be confirmed by appropriate examinations. In the absence of a visible cause of splenomegaly, first of all, the presence of infection should be eliminated, since it requires early treatment, unlike other causes of splenomegaly. The survey should be most complete in areas of high geographical spread of infection in the case when the patient has clinical signs of infection. It is necessary to obtain data from general analysis and blood cultures, and bone marrow studies. If the patient does not feel sick, there are no symptoms of the disease, except for the symptoms caused by splenomegaly, and there was no risk of infection, there is no need for a wide range of studies other than performing a general blood test, a peripheral blood smear, liver function tests, CT of abdominal organs cavity and ultrasound of the spleen. If suspected of lymphoma, the flow of peripheral blood flow-up is indicated.

Specific abnormalities in the analysis of peripheral blood may indicate the cause of the lesion (eg, lymphocytosis - for chronic lymphocytic leukemia, leukocytosis with the presence of immature forms - for other types of leukemia). The increased content of basophils, eosinophils, nucleated erythrocytes or erythrocytes in the form of a "falling drop" suggests the presence of a myeloproliferative disease. Cytopenia indicates hypersplenism. Spherocytosis suggests the presence of hypersplenism or hereditary spherocytosis. Functional hepatic tests will have deviations in stagnant splenomegaly with cirrhosis; an isolated increase in serum alkaline phosphatase indicates a possible liver infiltration as a result of myeloproliferative, lymphoproliferative disease or miliary tuberculosis.

Some other studies may be useful even in patients with no symptoms of disease. Whey protein electrophoresis identifies monoclonal gammopathy or a decrease in the level of immunoglobulins possible in the presence of lymphoproliferative diseases or amyloidosis; diffuse hypergammaglobulinemia presupposes the presence of a chronic infection (eg, malaria, Indian visceral leishmaniasis, brucellosis, tuberculosis), cirrhosis with congestive splenomegaly or connective tissue disease. An increase in serum uric acid level presupposes the presence of myeloproliferative or lymphoproliferative disease. An increase in the level of alkaline phosphatase of leukocytes indicates a myeloproliferative disease, whereas a decrease in its level may lead to the presence of chronic myelogenous leukemia.

If the examination showed no abnormalities except splenomegaly, the patient should be examined again within the 6 to 12 month interval or when new symptoms appear.

Splenomegaly increases splenic mechanical filtration and destruction of erythrocytes, as well as frequent leukocytes and platelets. Compensatory hyperplasia of the bone marrow is shown in those cell lines whose level is reduced in circulation.

[25], [26], [27], [28], [29],

Treatment of splenomegaly

Treatment of the disease is directed to the underlying disease. The enlarged spleen does not need treatment until severe hypersplenism develops. Patients with a spleen available for palpation or who have reached very large sizes should probably avoid contact sports in order to avoid the risk of rupture.