Splenomegaly: causes, diseases, surgery

Physicians refer to an enlarged spleen as splenomegaly. It can occur as a symptom of various diseases such as infections, disorders of blood formation or liver damage. Patients occasionally complain of a feeling of pressure in the left upper abdomen. The therapy depends on the underlying disease. The spleen may have to be removed. Read all about splenomegaly here!

Splenomegaly: Description

An enlarged spleen is a common symptom. It can occur with various diseases. These include infectious diseases, hereditary diseases, diseases of the blood or liver and many others. In order to better understand what the tasks and position of the spleen are in the body, an overview of the anatomy and physiology of the spleen follows.

Splenomegaly: anatomy and physiology of the spleen

The spleen is also called splenic or lien. As the largest lymphatic organ in the body, it is an important part of the immune system. It lies in the left upper abdomen, usually behind the lower ribs. It is normally about four centimetres thick, seven centimetres wide and eleven centimetres long. She’s encased in a pod. Its tasks include the interception and degradation of old and deformed blood cells and microorganisms occurring in the blood. In addition, immune cells mature in it. It is possible to live without a spleen. However, the risk of serious infections then increases.

Splenomegaly: Symptoms

Splenomegaly is itself a symptom of the disease, but it also causes other complaints. Therefore, in splenomegaly one must distinguish between symptoms caused by the enlargement of the spleen itself and those caused by the underlying disease.

Splenomegaly: symptoms of the underlying disease

Many different diseases can lead to splenomegaly. Depending on this basic disease, patients have symptoms. The following relationships can be assumed to point the way forward:

• For infectious diseases: Fever, fatigue, swelling of the lymph nodes
• For malignant diseases: Weight loss, night sweats, fever
• For blood disorders: Tiredness, weakness, paleness
• For liver damage: Jaundice, oesophageal bleeding, visible abdominal veins

Spleen enlargement: symptoms it causes

A pathological swelling of the spleen is usually palpable under the left costal arch. It can cause pain, for example when it presses on nerves or displaces other organs. If the spleen swells too much for the capsule surrounding it, it can rupture. The so-called splenic rupture is accompanied by severe pain in the left upper abdomen. This pain may radiate into the left shoulder.

Splenomegaly: causes and risk factors

The causes that can lead to splenomegaly are manifold. They can be divided into different groups.

Blood Diseases

There are benign and malignant diseases of the blood that can lead to splenomegaly. Among the benign ones are congenital defects of the red blood cells. These are:

• Sickle cell anaemia
• Thalassemia
• Hereditary spherocytosis
• Glucose-6-phosphate dehydrogenase deficiency

These diseases lead to a change in the structure of the erythrocytes. These get caught in the vessels of the spleen and are broken down there. As many erythrocytes accumulate, the spleen becomes larger to handle the numerous degradations.

Malignant diseases of the blood that cause an enlarged spleen are leukemias and lymphomas as well as myeloproliferative diseases such as osteomyelofibrosis or juvenile myelomonocytic leukemia.

Infections

Typical for a spleen enlargement is an infection with the Eppstein-Barr-Virus (EBV), which causes mononucleosis (Pfeiffer’s glandular fever). The cytomegalovirus (CMV) also frequently leads to splenomegaly in children. Other infectious diseases that can be associated with splenomegaly include

• bacterial sepsis
• Leishmaniasis
• Malaria
• Syphilis
• Typhoid
• Tuberculosis

Portal vein damage

If there is a flow obstruction in the portal vein, the blood accumulates back into the spleen (congestive spleen). Reasons for this may be:

• Heart failure
• Cirrhosis or fibrosis of the liver
• Portal vein thrombosis
• Budd-Chiari Syndrome

Associated conditions

Diseases that are associated with metabolic disorders can lead to spleen enlargement. These include, among others:

• Glycogen storage disease
• Niemann-Pick’s disease
• Gaucher’s disease
• Mucopolysaccharidoses

Immunological diseases

Various immunological diseases can be the cause of splenomegaly. These include, among others:

• Chediak Higashi Syndrome
• Kawasaki Syndrome
• Histiocytoses
• Chronic granulomatosis
• Autoimmune Lymphoproliferative Syndrome (ALPS)

Other causes

Other causes of an enlarged spleen can be space requirements in the organ itself. These include distant metastases of malignant tumours, haemangiomas or hamartomas of the spleen. Lymphomas and leukemias can also infiltrate the spleen tissue.

In rare cases, spleen swelling can also occur in collagenoses such as systemic lupus erythematosus, Still’s disease or juvenile rheumatoid arthritis. Splenomegaly is also possible in sarcoidosis.

Splenomegaly: examinations and diagnosis

If you feel ill and feel pain in your left upper abdomen, consult your family doctor. He can make a diagnosis of splenomegaly and order further investigations to find the cause. First he will ask you in detail about your medical history (anamnesis). He may ask you the following questions:

• Have you suffered from an infection recently?
• Do you suffer from a chronic or malignant disease?
• Do you have a fever?
• Have you lost weight unintentionally recently?
• Do you wake up at night in a sweat?

Splenomegaly: physical examination

A large spleen can be palpated under the left costal arch during the physical examination. The patient lies on the right side. The examiner stabilizes the patient with the left hand while palpating the left upper abdomen with the right hand. Normally the spleen should not be palpable. If your doctor palpates it, you have splenomegaly. He can then confirm this in an ultrasound examination in which he measures the spleen. In addition, he may find indications of liver damage or disease of the portal vein in ultrasound.

In addition, your doctor will look for swollen lymph nodes and signs of liver damage during the physical examination. These can be a yellowing of the skin, redness on the palms of the hands, visible veins of the abdominal wall or small, star-like haemangioma on the décolleté.

Splenomegaly: Further diagnostics

If your doctor has diagnosed splenomegaly, further examinations are necessary to find causes for the enlarged spleen. At first blood should be taken from the patient. In the laboratory they are examined in it:

• Blood count and blood smear (number of red and white blood cells and platelets including a list of the different types of white blood cells and the number of young red blood cells)
• Blood sedimentation rate
• Evidence of liver damage: Transaminases (ALAT, ASAT), Bilirubin
• Immune parameters (C-reactive protein, antinuclear antibodies, rheumatoid factors, Coombs test, electrophoresis)
• Sign for virus infections

According to the results of the blood test, certain diseases can be ruled out as the cause of splenomegaly, while others can be considered as probable causes. Further diagnostic steps can then be taken, such as an X-ray of the chest, a computer tomography of the abdomen or a bone marrow biopsy.

Splenomegaly: treatment

Splenomegaly is usually a symptom of another underlying disease. Accordingly, once the basic disease has been diagnosed, it must be treated. With effective therapy, splenomegaly often also disappears.

Surgical removal of the spleen (splenectomy) is also a possible treatment for splenomegaly. This is necessary if the spleen capsule ruptures due to the increase in size or if the spleen becomes overactive (hypersplenism). Splenectomy should be used as a last resort, as it carries the risk of serious infectious diseases. This is known as overwhelming post-splenectomy infection (OPSI). Especially encapsulated bacteria such as pneumococci or meningococci can no longer be fended off sufficiently.

Splenomegaly: course of disease and prognosis

The course of the disease and the prognosis of splenomegaly depend strongly on the underlying disease causing the swollen spleen. Without treatment of the cause, the spleen can swell so much that its capsule ruptures. Depending on the severity of the tear, surgery may be required. If the haemostasis is successful, spleen preserving surgery can be performed. If the bleeding cannot be stopped, the spleen must be removed immediately.

Another complication that can lead to a splenectomy is the so-called hypersplenism. It represents a hyperactivity of the spleen. It then removes more blood cells than necessary (excessive phagocytosis). Since the bone marrow cannot produce the now missing blood cells fast enough, pancytopenia develops. By this doctors understand that there are too few red and white blood cells and platelets in the blood. This leads to weakness, susceptibility to infection and diffuse bleeding. In this case the spleen should be removed in case of splenomegaly.