Hepatosplenomegaly: what is it, how to treat?

The simultaneous pathological enlargement of such visceral organs as the liver (in Latin – hepar) and spleen (in Greek – splen) is defined in medicine as hepatosplenomegaly. In the section of symptoms and signs of ICD-10, its code is R16.2.

Epidemiology

Hepatosplenomegaly is not a nosological form and refers to symptoms, and usually there are no official statistics on the manifestation of symptoms, but they can be recorded in individual clinical studies.

Thus, it is known that in approximately 30% of cases, an enlarged spleen is caused by hepatomegaly; in mononucleosis, hepatosplenomegaly is observed in 30-50% of cases, and in typhoid fever, an enlarged liver with severe jaundice and, to a lesser extent, swelling of the spleen are observed in a third of patients. In acute hepatitis A, in more than 65% of patients, only the liver is enlarged, while the incidence of hepatosplenic syndrome does not exceed 15-18%. [ 1 ]

According to WHO, chronic hepatosplenomegaly in children is common in rural areas of sub-Saharan Africa, and the two most common infections leading to the condition are malaria and schistosomiasis.

Causes hepatosplenomegaly

A wide variety of diseases can cause hepatosplenomegaly syndrome, which is also called hepatosplenic (from Latin lienem – spleen).

First of all, the causes of enlargement of the liver and spleen are associated with hepatotropic and systemic infections affecting them.

This is hepatosplenomegaly in hepatitis A, B, C, D and E, which is known to be of viral origin. The liver hepatocytes are damaged by viral DNA replication and the body's immune response to their antigens.

Hepatosplenomegaly in mononucleosis is associated with damage to the spleen macrophages and liver Kupffer cells by the herpes virus type IV (Epstein-Barr virus). In this case, there is an increase in lymph nodes (often generalized) - with a significant increase in the level of lymphocytes in the blood - and inflammatory edema of the spleen and liver, defined by doctors as prolonged lymphadenopathy, hepatosplenomegaly in combination with absolute lymphocytosis.

Inflammation of the liver with subsequent hepatosplenomegaly can also occur with infection with the herpes virus type V - cytomegalovirus; in particular, hepatosplenomegaly is noted with pneumonia caused by cytomegalovirus infection.

Hepatosplenomegaly is possible with adenovirus infection, which causes acute inflammation of the respiratory system, and serotypes F40-41 - adenovirus gastroenteritis (especially in children). Pathogenesis is associated with the ability of DNA adenoviruses to penetrate the lymph nodes and systemic bloodstream, causing intoxication. Read - Symptoms of adenovirus infection.

Hepatosplenomegaly is observed as one of the late symptoms in salmonellosis (in case of infection with intestinal bacteria Salmonella enterica); in case of infection with helminths of the Schistosomatidae family and development of schistosomiasis; in case of liver echinococcosis, visceral form of leishmaniasis, in patients with opisthorchiasis.

Hepatosplenomegaly almost always develops with malaria, the causative agent of which is the malarial plasmodium (Plasmodium malariae). Infectious disease specialists explain the mechanism of development by the fact that the plasmodium infects erythrocytes and enters the liver with the bloodstream, in the cells of which it multiplies. However, painful enlargement of the liver and spleen begins in the erythrocytic phase. With this disease (often chronic, with relapses), cyclic fever is observed in combination with hepatosplenomegaly, nausea and vomiting; due to the death of erythrocytes (hemolysis), yellowing of the skin may be observed. [ 2 ]

Hepatosplenomegaly in typhoid fever is also caused by hematogenous spread of Salmonella typhi enterobacteria affecting regional lymph nodes, liver and spleen cells, which cause focal infiltration of mononuclear cells circulating in the blood with the formation of typhoid granulomas in organ tissues and their inflammatory proliferation.

How hepatosplenomegaly occurs in tuberculosis (acute disseminated form) is described in detail in the publications:

• Tuberculosis and liver disease
• Tuberculous hepatitis
• Liver cirrhosis and hepatosplenomegaly see - Secondary biliary cirrhosis of the liver.

And with chronic alcoholism or severe poisoning, cirrhosis of the liver of toxic origin and hepatosplenomegaly develop.

A number of hematological diseases lead to enlargement of the liver and spleen. More than 70% of patients have lymphadenopathy and hepatosplenomegaly in leukemia, including acute leukemia (lymphoblastic leukemia), when leukemic cells - mutated immature white blood cells (blasts) - penetrate and accumulate in the lymph nodes, liver and spleen.

Chronic myeloid leukemia or chronic myeloid leukemia and hepatosplenomegaly are observed when bone marrow myeloid cells (myeloblasts) accumulated in the spleen and liver enter the bloodstream. However, as experts say, with this disease, an enlargement of the spleen most often occurs - splenomegaly. [ 3 ]

Hepatosplenomegaly develops with anemia, primarily sickle cell and pernicious, and in children - with congenital hemolytic anemia and thalassemia. Jaundice and hepatosplenomegaly (or the formation of multiple nodular formations in the parenchyma of the liver and spleen), characteristic of hemolytic anemia, are often similar to liver diseases. [ 4 ]

Doctors do not exclude the possibility of hepatosplenomegaly in heart failure caused by serious cardiac diseases with pathological changes in the structure of the heart, circulatory disorders and ischemia.

Transient enlargement of the spleen (transient splenomegaly) is possible in severe cases of acute pancreatitis - with stenosis of the splenic vein and/or simultaneous acute inflammation of the spleen. But pancreatitis and hepatosplenomegaly can be observed in patients with a reactive type of inflammation of the pancreas, as well as its rare form - autoimmune.

Risk factors

Chronic diseases of the hepatobiliary system and all the above-mentioned diseases and pathologies are risk factors for the development of hepatosplenomegaly syndrome. Experts include radiation exposure, alcohol dependence, and decreased immunity among such factors – not only in acquired immunodeficiency syndrome, but even during pregnancy.

Infection-related hepatosplenomegaly may occur more easily during pregnancy, since physiological immunosuppression puts pregnant women at increased risk of infection. In addition, in some cases, pregnancy causes partial blockage of blood flow in the hepatic portal vein with increased pressure in it - portal hypertension - in combination with hepatosplenic syndrome.

Symptoms hepatosplenomegaly

In the clinical picture of diseases leading to enlargement of the liver and spleen or accompanied by hepatosplenic syndrome, its first signs - a constant feeling of heaviness on the right side in the hypochondrium, pain on palpation, weight loss, attacks of weakness - do not appear immediately.

In addition, at the first stage – with a mild degree of organ enlargement – blood tests may show a decrease in hemoglobin and leukocytes.

As the liver (by 20-40 mm) and spleen (by 10-20 mm) further increase, moderate hepatosplenomegaly is observed, and later, pronounced, significant hepatosplenomegaly, in which the increase in the size of the liver exceeds 40 mm, and the spleen - 20 mm.

And depending on this, symptoms such as abdominal pain in the upper right corner; nausea, vomiting and bloating; dyspeptic disorders; dry and pale skin, and in liver disease and amyloidosis - jaundice with itching; pain in muscles and joints; enlarged lymph nodes. In some cases, pain in the left hypochondrium and hyperthermia may be observed.

Hepatosplenomegaly and portal hypertension are etiologically related and develop with hepatitis, cirrhosis, congenital fibrosis, tumors and parasitic infections of the liver, as well as subleukemic myelosis (myelofibrosis). [ 5 ] Early onset of portal hypertension signs is usually characteristic of damage to the portal and splenic veins. More information in the material - Portal Hypertension - Symptoms.

Often, with the same diseases, against the background of portal hypertension and obliterating thrombosis of the liver (Budd-Chiari syndrome), hepatosplenomegaly and ascites (accumulation of fluid in the abdominal cavity) are observed.

Hepatosplenomegaly and thrombocytopenia (low platelet count in the blood) is a sign of increased functional activity of the spleen (hypersplenism) with destruction of these blood cells. Most often, this occurs in patients with cirrhosis. For more information, see - Thrombocytopenia and platelet dysfunction

Black stool – melena in the presence of hepatosplenomegaly – is characteristic of congenital liver fibrosis and tyrosinemia in newborns.

The appearance in infants of symptoms such as generalized exfoliative dermatitis with erythroderma, enlarged lymph nodes, alopecia, and hepatosplenomegaly is observed in genetically determined Omenn syndrome and primary immunodeficiency.

If an enlarged liver and spleen (with their compaction) in a newborn is accompanied by focal alopecia, macular-nodular rash (including on the mucous membranes), deformation of the tubular bones of the skeleton, then this is evidence of the presence of congenital syphilis.

In older children, hepatosplenic syndrome and hair loss may be part of the clinical manifestation of autoimmune hepatitis. And in adults, this combination of symptoms occurs in such an autoimmune disease as sarcoidosis.

Hepatosplenomegaly in children

Hepatosplenomegaly is more common in children than in adults. In childhood, enlargement of the liver and spleen may be associated with hepatitis and the infections listed above. Thus, hyperemia of the skin and mucous membranes, hepatosplenomegaly are characteristic of infectious rubella caused by Rubella virus, or Syphilis congenita - congenital syphilis.

The development of hepatosplenic syndrome is observed in acute leukemia, congenital liver fibrosis and thrombosis of the portal vein, hereditary spherocytosis (Minkowski-Chauffard disease), and hereditary familial hemorrhagic angiomatosis.

One of the symptoms of hemolytic disease of the newborn, ductular hypoplasia (Alagille syndrome), hereditary cerebrosidosis (glucocerebrosidase gene mutations) - Gaucher disease, as well as hereditary sphingolipidosis (Niemann-Pick disease) is hepatosplenomegaly in newborns and children of the first year of life. Read also - Hepatomegaly in children [ 6 ], [ 7 ]

Genetic syndrome of connective tissue dysplasia with hepatosplenomegaly in children may be caused by a mixed disease of connective tissue of autoimmune origin, in which the body produces antibodies to U1 ribonucleoprotein (U1-RNP). The disease manifests itself with symptoms characteristic of systemic lupus erythematosus, scleroderma and polymyositis.

The development of hepatosplenomegaly syndrome occurs in children with congenital (inherited mutations of genes encoding certain enzymes) disorders of carbohydrate metabolism – mucopolysaccharidoses, which are lysosomal storage diseases, in this case – accumulation of glycosaminoglycans (mucopolysaccharides). Among these diseases are:

• Hurler syndrome (Hurler-Scheie) or mucopolysaccharidosis, type I;
• Sanfilippo syndrome - mucopolysaccharidosis type 3;
• Maroteaux-Lamy syndrome - mucopolysaccharidosis type VI;
• Sly syndrome - mucopolysaccharidosis type VII.

In infants and young children, enlargement of the liver and spleen (with intermittent fever) may result from tyrosinemia, an inborn error of metabolism in which inherited mutations in various genes prevent the catabolism of the amino acid tyrosine.

Two such serious symptoms as hepatosplenomegaly and thrombocytopenia appear in adult patients with T-cell non-Hodgkin's lymphomas and some other malignancies, vascular-follicular hyperplasia of the lymph nodes (Castleman disease), and in children - with hereditary Niemann-Pick disease, as well as with a disease of the heart muscle - dilated cardiomyopathy.

Complications and consequences

Bleeding, blood in the stool and vomit, functional liver failure and hepatic encephalopathy (in severe cases – with cytotoxic cerebral edema) are considered the most common complications and consequences associated with hepatosplenomegaly syndrome.

Diagnostics hepatosplenomegaly

In addition to palpation and percussion examination of the liver and spleen, a complete medical history (including family members) and patient complaints, diagnosis of diseases leading to hepatosplenomegaly includes a wide range of laboratory and instrumental studies.

The following tests are required: general clinical and biochemical blood test, for bilirubin content in the blood; for serum levels of total protein, albumin, C-reactive protein, aminotransferases, alkaline phosphatase, iron; for blood clotting; for the presence of IgM and IgG antibodies (to hepatitis viruses, LSP, LKM, etc.) - the so-called blood test for liver function tests. The activity of some enzymes in the blood plasma is determined. A general urine test is taken, as well as a coprogram - to detect intestinal bacteria and helminths. An aspiration biopsy of the liver, lymph nodes or bone marrow may be required.

Instrumental diagnostics are performed, in particular, ultrasound of the spleen and liver, Dopplerography of the liver vessels. But more informative than ultrasound examination is the CT picture of hepatosplenomegaly, visualized by computed tomography of the abdominal cavity with contrast, CT and scintigraphy of the spleen and liver. [ 8 ]

In this case, hepatosplenomegaly, diffuse changes in the liver and reactive changes in the pancreas may be detected. In most cases, patients with liver cirrhosis, autoimmune hepatitis and fatty hepatosis have hepatosplenomegaly and diffuse changes in the liver. Morphological changes in the liver and spleen, such as hyperplasia of loose connective tissue cells (histiocytes), death of hepatocytes and fibrosis are often observed.

Differential diagnostics help to establish the true cause of enlarged liver and spleen. [ 9 ]

Treatment hepatosplenomegaly

In the presence of hepatosplenomegaly, treatment is carried out for the diseases that led to the development of this symptom.

It is clear that the therapeutic methods used and the medications prescribed depend on the underlying diagnosis. [ 10 ]

Read more:

• Hepatomegaly
• Enlarged liver
• Medicines for the treatment and restoration of the liver
• How to treat hepatitis, see the publication - Acute viral hepatitis

In rare cases, surgical treatment may also be used – removal of the spleen and part of the liver. Patients with severe and life-threatening conditions associated with hepatosplenomegaly may require a liver transplant.

Depending on the diagnosis, a diet is prescribed, for example:

• Diet for Hepatitis B
• Diet for chronic hepatitis
• Diet for liver cirrhosis

In case of mucopolysaccharidoses, diet therapy is of an elimination nature (excluding lactose and galactose).

Prevention

The causes of hepatosplenomegaly are varied, so it is not always possible to prevent it. And prevention of hepatosplenic syndrome, first of all, involves combating the causes of liver and spleen diseases.

Forecast

The specific outcome and prognosis of hepatosplenomegaly depends on many factors, including its etiology, severity, and treatment.