Causes, Symptoms and Treatments Options for Spleen Lymphoma

Spleen lymphoma is exceptionally rare type of primary cancer of spleen. It is also called splenic marginal zone lymphoma. A lymphoma is cancer of lymphocytes; a type of white blood cells which protect the body against any type of infection. Lymphoma develops when the lymphocytes multiply uncontrollably. The lymph fluid present in the lymphatic system carries lymphocytes.

Spleen is a part of lymphatic system. Lymphomas are of two types, non Hodgkin’s lymphoma and Hodgkin’s lymphoma. Spleen lymphoma is B-cell non Hodgkin’s lymphoma. Spleen lymphoma usually develops after the age of 50 years more commonly in men. In the early stage, patient may not present with any symptoms, but later on the disease may lead to enlarged spleen, pain in left upper quadrant of abdomen, easy bruising, fatigue and anemia. The cause of splenic lymphoma remains unknown. But certain medical conditions and exposure to environmental toxins is the likely etiology. Treatment consists of wait and watch approach in asymptomatic patients. In case of symptom presentation, spleen is surgically removed. Early diagnosis and treatment has good prognosis.


The cause of spleen lymphoma is not known.

However, there are various likely possibilities that are suspected to trigger uncontrollable multiplication of B cell lymphocytes in the spleen. These factors include;

  • People suffering from infections such as Epstein Barr virus infection, Hepatitis C virus, repeated H. pylori infection, HIV infection, systemic lupus and other autoimmune diseases such as psoriasis and rheumatoid arthritis.
  • Smoking
  • Genetic disorders such as Down’s syndrome
  • Exposure to environmental toxins, insecticides and pesticides, chemicals such as benzene.
  • Exposure to radiation
  • Family history of splenic cancer
  • People over the age of 50 and above
  • Male are more likely to develop as compared to females.
  • Being Caucasian

Symptoms of Splenic Lymphoma:

Majority of patients suffering from spleen cancer do not experience any symptoms. In many cases the disease is found accidentally either while the doctor is examining the patient’s abdomen for some other condition or the abnormal lymphocytes are detected in the blood. During the examination the doctor may find extremely enlarged spleen. Spleen in located in the left upper quadrant of the abdomen. In a normal healthy patient, spleen is not palpable physical examination of abdomen. In medical terms an enlarged spleen is called splenomegaly. Symptoms develop when the spleen is extremely enlarged in size. It may compress the nearby structures and organs. Pain in left upper side of abdomen may be one of the symptoms. Besides, patient may also develop anemia, fatigue due to anemia, bruising with slight injury, fever, loss of weight, intense itching, edema in legs, frequent infection, low platelet count in blood.

Diagnosis and Treatment:

Although spleen lymphoma is a rare type of cancer, it is imperative to differentiate spleen lymphoma with other types of splenic benign lesions. Diagnosis is also required to differentiate types of spleen lymphoma (B-cell lymphoma) so that appropriate treatment can be started. Various test for spleen lymphoma include; CT guided spleen biopsy, endoscopic ultrasound fine needle aspiration (EUS-FNA) is nowadays used for the purpose of sampling spleen tumors.

Once spleen lymphoma is confirmed with these tests, treatment module is decided on whether the patient is asymptomatic or symptomatic. Majority of patients do not manifest with spleen lymphoma symptoms. In such cases physicians usually monitor the patient regularly and follow wait and watch approach. If certain disease is found to be an underlying cause such as hepatitis C, HIV, GB syndrome, etc, then specific treatment measures are taken to deal with this underlying etiology. Once the underlying cause is addressed and treated, treatment may not be needed for spleen lymphoma.

However, if the symptoms are severe and if the blood count is low, than surgical removal of spleen may be required for relief of the symptoms. Surgery for spleen removal is called splenectomy. It is a major surgery which is carried out if patient is fit for surgery or else other alternative measures such as chemotherapy, radiation or immune therapy are used.