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Lymphoma of Eye

Last updated Nov. 3, 2018

Approved by: Krish Tangella MD, MBA, FCAP

DoveMed.com

Lymphoma of Eye is an uncommon lymphoma, which is generally observed in elderly adults. This is a microscopic pathology image showing marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type (MALT-type lymphoma, MALT lymphoma).


What are the other Names for this Condition? (Also known as/Synonyms)

  • Ocular Lymphoma

What is Lymphoma of Eye? (Definition/Background Information)

  • Lymphoma of Eye is an uncommon lymphoma, which is generally observed in elderly adults. It is also known as Ocular Lymphoma. The condition may affect the conjunctiva, inside structure of eye, eyeball, or tissues around the eye orbit
  • The condition may be primary or secondary:
    • Primary Lymphoma of Eye (also known as Primary Intraocular Lymphoma or PIOL): This type of lymphoma first involves the eye and later can involve other parts of the body including the lymph nodes and bone marrow
    • Secondary Lymphoma of Eye: This type of lymphoma involves other parts of the body first, such as peripheral blood, lymph nodes, bone marrow, and other organs; eye involvement occurs later
  • Lymphoma of Eye can either be a B-cell lymphoma or a T-cell lymphoma. B-cell lymphomas are far more common than T-cell lymphomas
  • There are various (histological) subtypes of Ocular Lymphomas and some of these include:
    • Diffuse large B-cell lymphoma of eye or ocular adnexal DLBCL
    • MALT lymphoma of eye or ocular MALT lymphoma
  • Lymphoma of Eye may be associated with autoimmune disorders. The cause of the condition is generally unknown. It is generally noted to be present with lymphoma affecting the central nervous system
  • It can cause blurred vision, redness of the eyes, burning sensation, and other general signs and symptoms, such as fever, weight loss, and appetite loss. In case of severe complications, it may result in vision loss
  • Chemotherapy, surgery, radiation therapy, and other treatment measures may be used for treating Lymphoma of Eye based on the assessment of the physician. Additionally, treatment for any underlying condition will also be considered by the healthcare provider
  • The prognosis depends on many factors including the subtype and stage of lymphoma, progression of the condition, response to treatment, and overall health of the individual. In general, the prognosis of Lymphoma of Eye is guarded

General information on lymphoma and lymphocytes:

  • Lymphoma is a type of cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
  • Lymphocytes are the main white blood cells found in the lymph, which is the fluid of the lymphatic system; just as blood is the fluid of the circulatory system. Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells
  • Lymph results from filtration of blood as it travels to and from tissues. Lymph is colorless because it lacks red blood cells; instead, it contains lymphocytes. It is central to the immune system
  • There are 3 different kinds of lymphocytes:
    • T-lymphocytes or T cells: They help combat infections and abnormalities within the cells (cell-mediated immunity). They fight viruses and cancerous cells
    • B-lymphocytes or B cells: They produce antibodies that are bodily defense proteins, which target foreign invaders outside the cells (humoral immunity). They fight bacterial cells, cell fragments, and other immunogenic elements
    • Natural killer cells or NK cells: They perform diverse functions related to both cell-mediated and humoral immunity. They also scout for cancer cells, a process called immune surveillance

Who gets Lymphoma of Eye? (Age and Sex Distribution)

  • Lymphoma of Eye occurs infrequently. It is generally seen in older men and women
  • Both males and females can be affected
  • It can occur worldwide and all races and ethnic groups may be affected

What are the Risk Factors for Lymphoma of Eye? (Predisposing Factors)

No specific risk factors have been identified for Lymphoma of Eye. However, the condition is known to be associated with the following factors:

  • Autoimmune disorders
  • Chlamydia psittaci infection of the eye. It is a bacterial infection that commonly affects birds
  • Lymphoma of the central nervous system (CNS)
  • Advanced age; older individuals commonly have a higher risk
  • Individuals with weak immune system (due to various health conditions)

Besides the above, the following general factors may contribute towards lymphoma formation and development:

  • Family history of immune disease
  • The presence of any systemic disease
  • Smoking
  • Exposure to radiation and industrial chemicals
  • Chemotherapy
  • Viruses (in some rare cases); Epstein-Barr virus infection
  • X-ray, CT scan exposure
  • Profession involving radiation exposure, which may include nuclear plant workers, pilots, astronauts, etc.
  • Certain medications and drugs

International Prognostic Index: According to some scientists, the International Prognostic Index may not be very helpful in evaluating Ocular Lymphoma. However, some scientists believe that it is helpful in some cases, to determine the prognosis.

The International Prognostic Index, for aggressive non-Hodgkin lymphoma, lists a few factors that determine the overall risk:

  • Age over 60 years
  • Elevated level of serum lactate dehydrogenase - LDH (a type of enzyme)
  • Performance status, i.e. the overall health condition of the individual, which could range from being fully active (low risk) to being completely disabled (very high risk)
  • Individual, who have already suffered from lymphoma, or other types of blood cancers, may have a relapse or a recurrence
  • Presence of an immunodeficiency syndrome, like AIDS, is a high risk factor
  • Those infected with Epstein-Barr virus are also prone to this lymphoma type

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Lymphoma of Eye? (Etiology)

Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. When under certain circumstances, the lymphocytes grow and multiply abnormally, it leads to a condition called as lymphoma, which is a most common type of cancer. There are 2 types of lymphoma:

  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma

The cause of Lymphoma of Eye is unknown. There may be certain genetic defects, such as translocation, which is a juxtaposition of regions of the chromosomes. This may result in:

  • Change of regulatory elements of certain cancer-causing genes called as oncogenes, which can lead to increased production of their mRNA (overexpression), thus increasing their protein levels
  • Exchange of protein coding regions of gene, giving rise to new proteins that can stimulate the inappropriate growth of cells

It is believed that the abnormal development of lymphocytes gives rise to cancerous cells leading to the formation of this condition. Nevertheless, how this occurs and the factors that cause it remain under investigation.

What are the Signs and Symptoms of Lymphoma of Eye?

The signs and symptoms of Lymphoma of Eye depend on whether it is a primary condition or a secondary condition. In a majority of primary lymphomas, if the tumor is small, it may be usually asymptomatic and the individuals will not have any significant symptoms. The symptoms also depend upon the extent of lymphoma involvement in other parts of the body.

The lymphoma may affect any part of the eye, such as the conjunctiva (conjunctival lymphoma), inside structure of the eye (intraocular lymphoma), globe of the eye (the eyeball), or tissues around the eye orbit. The signs and symptoms of Ocular Lymphoma may include:

  • Burning sensation in the eye; watery eye
  • Foreign body sensation in the eye
  • Redness of the eyes
  • Blurry vision
  • Unintentional weight loss; changes in appetite
  • Fatigue and weakness, headache
  • Anemia (low red blood cell count)
  • Frequent infections
  • Low blood pressure
  • Generally, the involvement of both eyes may be seen (bilateral condition)

When organs other than the eyes are involved, then the signs and symptoms may be based on the specific organs that are affected.

How is Lymphoma of Eye Diagnosed?

Lymphoma of Eye diagnosis is generally performed by obtaining biopsy samples from the affected region and examining them under a microscope to detect the cancerous cells. There are other tests and procedures that could help in the diagnosis and these include:

  • A thorough physical examination and a complete medical history, which is very important
  • A thorough eye examination by an eye specialist
  • Blood tests that may include:
    • Complete blood cell count (CBC) blood test
    • Absolute lymphocyte count on peripheral blood
    • Liver function blood test (LFT)
    • Lactate dehydrogenase (LDH) blood test
  • Tissue biopsy from the affected region:
    • A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis
  • Radiological imaging may be performed specific to location of the involved organ, and to determine the extent of lymphoma in the body including:
    • Ultrasound scan of head and neck region
    • Computerized tomography (CT) scan of head and neck region
    • Vascular radiological studies
    • Whole body bone scan
    • Whole body CT-PET scans to determine how far the lymphoma has spread, by checking the size and metabolic rate (a reflection of uncontrolled growth) of lymph nodes, throughout the body. This can also help determine, if the cancer has spread to other organ systems
    • Brain MRIs are used if neurological symptoms are present, which can help determine if the cancer has spread to the brain, or to tissues that cover the brain
  • Bone marrow aspiration and biopsy is performed and sent to a laboratory for a pathological examination, to determine if the bone marrow is involved. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely electron microscopic studies. However, a bone marrow biopsy is not needed in the early stages of the condition
  • Flow cytometry to identify cells as they flow through an instrument, called a flow cytometer. Flow cytometry measures the number and percentage of cells in a blood sample, and cell characteristics such as size, shape, and the presence of biomarkers on the cell surface. This method helps to sub-classify the condition and also to detect residual levels of disease after treatment. This tool can help in diagnosing relapse and restart treatment as needed
  • Fluorescence in situ hybridization (FISH): It is a test performed on the blood or bone marrow cells to detect chromosome changes (cytogenetic analysis) in blood cancer cells. The test helps in identifying genetic abnormalities that may not be evident with an examination of cells under a microscope
  • Immunophenotyping to identify a specific type of cell in a sample, which can help determine the best treatment course to be followed
  • Polymerase chain reaction (PCR): It is used to measure the presence of certain biomarkers in blood or bone marrow cells. The test is ultrasensitive and detects extremely low amounts of biomarkers remaining in blood, which can be missed by cytogenetic methods, such as FISH, karyotype, or flow cytometry. PCR allows a more sensitive follow-up of patients in remission and can help determine whether additional treatment is necessary
  • Exploratory laparoscopy (diagnostic laparoscopy) may be required, if gastrointestinal symptoms are present. In this procedure, the abdomen is examined using a minimally-invasive technique, and a tissue biopsy and tissue for culture obtained. Minimally-invasive approaches help decrease complications and the length of stay at the hospital. A diagnostic laparoscopy is also helpful in staging of the tumor. Nevertheless, this procedure is not very much used

Note: Differential diagnoses, to eliminate other tumor types are often considered, before arriving at a definitive diagnosis.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Lymphoma of Eye?

The complications due to Lymphoma of Eye may include:

  • Involvement of local and distant organs: It can lead to systemic or disseminated disease in some cases
  • Loss of function of the organ/area to which cancer has spread due to systemic involvement
  • Irreversible damage to the cornea resulting in permanent vision loss
  • Complications arising due to the presence of CNS lymphoma that is typically associated with Ocular Lymphoma
  • Weakened immune system (or immunosuppression) can be a complication, which can become more severe during treatment. Due to this, individuals are more vulnerable to infections; there is an increased risk of developing serious complications from such infections
  • Occasionally, the tumor can transform into a more aggressive form or subtype of lymphoma

There may be complications related to chemotherapy used in treating the condition, which may include:

  • Side effects such as dizziness, vomiting, appetite loss, mouth ulcers, and hair loss
  • By damaging healthy cells, the individual is more open or vulnerable to secondary infections
  • The treatment can also cause infertility in men and women. Hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy

The treatment measures can also give rise to secondary cancers, such as skin cancer.

How is Lymphoma of Eye Treated?

A combination of measures may be used to treat Lymphoma of Eye. The treatment also depends upon the stage, overall health, age, and subtype of lymphoma.

  • Antibiotic therapy for Chlamydia psittaci infection
  • Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and can be used in patients, for all stages of the tumor
    • There can be severe side effects including fatigue, nausea, hair loss, anemia, high risk of infection, and drug-specific reactions
    • Many lymphomas can be resistant to chemotherapy. It can also damage healthy cells
    • Chemotherapy can be administered as a pill, liquid, shot, or intravenously

Note: Men and women in child-bearing age would greatly benefit from counseling regarding fertility issues. Some chemotherapy agents can cause infertility in both men and women. There can be permanent damage to the testicles and ovaries, harming their ability to produce sperms or ova. In men, sperm banking can be considered before initiating therapy. In women, in many cases, due to urgency of starting chemotherapy, it is often difficult to perform ovum banking. However, if there is sufficient time prior to chemotherapy, ovum banking may be performed. The healthcare provider may help assess the risk-benefit analysis, depending upon each individual’s specific circumstances.

  • Surgical removal of the lymphoma from the eye
  • Radiation: Radiation therapy is the use of high-energy radiation waves to kill cancer cells, by destroying their DNA
    • This treatment modality is generally used for early stage lymphomas. It is most commonly used in combination with chemotherapy
    • The radiation may be administered by a machine placed outside the body, or by placing a radioactive material inside the body
    • The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of cancer later in life, and risk of heart disease
    • Radiation can damage healthy cells in addition to cancer cells, causing further complications
  • Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics, may be used as supportive therapy. In combination with other treatment measures, these can help combat the symptoms of immunodeficiency
  • Undertaking treatment of underlying infection/disorder, as warranted
  • If Ocular Lymphoma is not fully responsive to treatment, or if the chance of recurrence is high, then bone marrow transplantation or stem cell transplantation can be considered
  • In order to prevent infections because the immune system is weakened by the lymphoma or by its treatment, the patient is kept in an isolated ward and treated with appropriate antibiotics
  • Nowadays targeted therapies are being developed that can selectively kill the cancer cells. Many of them are in the stage of clinical trials
  • Clinical trials: There may be some newer treatment options, currently on clinical trials, which can be considered for some patients depending on their respective risk factors

Your healthcare provider will determine the best course of treatment depending on your individual circumstances. Also, follow-up care with regular screening and check-ups are important post-treatment.

How can Lymphoma of Eye be Prevented?

Currently, it is not possible to prevent Lymphoma of Eye. However, controlling certain factors may help lower one’s risk for the condition.

  • Healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help decrease its risk
  • Avoiding smoking
  • Undertaking appropriate and early treatment for bacterial infection that affect the eye and other autoimmune disorders, if any
  • Using appropriate protective gear while working with x-rays and other radioactive source
  • In order to avoid a relapse, or be prepared for a recurrence, the entire diagnosis, treatment process, drugs administered, etc. should be well-documented and follow-up measures initiated

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Lymphoma of Eye? (Outcomes/Resolutions)

  • Lymphoma of Eye is a slow-growing malignancy with a generally good prognosis with early diagnosis and treatment
  • The prognosis is mainly dependent upon the tumor stage and lymphoma subtype. Also, primary lymphomas have better prognoses than secondary or recurrent lymphomas
  • Also, this lymphoma type is generally associated with the presence of lymphoma affecting the central nervous system (brain or spinal cord). This can make the outcome of the condition unpredictable
  • In general, the prognosis depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Individuals with bulky disease have a poorer prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) - it is a rare option
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
    • Progression of the condition makes the outcome worse (progressive Ocular Lymphoma)
  • The combination chemotherapy drugs used, may have some severe side effects (such as cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Tolerance to the chemotherapy sessions is a positive influencing factor
  • Progression to bone marrow failure is usually associated with short survival

Additional and Relevant Useful Information for Lymphoma of Eye:

  • Treatment for Lymphoma of Eye can cause physical and emotional distress; supportive care and encouragement, help positively and can bring a measure of relief to the patients

The following article link will help you understand leukemia and lymphoma (blood cancer):

https://www.dovemed.com/diseases-conditions/leukemia-and-lymphoma/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: March 7, 2016
Last updated: Nov. 3, 2018