Lymphoproliferative disorders are a group of diseases characterized by the uncontrolled production of white blood cells called lymphocytes.
Lymphocytes can be divided into B cells, T cells, and natural killer cells:
- B cells produce proteins called antibodies that signal for other cells in your immune system to attack foreign invaders.
- T cells destroy cells that have been taken over by foreign invaders or have become cancerous.
- Natural killer cells contain enzymes that can kill cancer cells or cells infected with foreign invaders.
Lymphoproliferative disorders develop due to immune system dysfunction.
These disorders can be cancerous or noncancerous. Cancerous lymphoproliferative disorder can broadly be classified into
- B-cell lymphocytic disorders
- T-cell chronic lymphoproliferative disorders
Each of these categories can be further divided into subcategories.
The specific symptoms, treatment, and outlook of lymphoproliferative disorders depend on which type you have.
Lymphoproliferative disorders are a group of conditions that lead to:
- monoclonal lymphocytosis, abnormally high levels of lymphocytes in the blood
- lymphadenopathy, swollen lymph nodes
- bone marrow infiltration, the disruption of normal blood cell production in bone marrow
Many conditions fall under the umbrella of lymphoproliferative disorders. Here are some examples:
B-cell lymphoproliferative disorders
B-cell lymphoproliferative disorders include:
|B-cell chronic lymphocytic leukemia||A subtype of CLL where cancer develops slowly in cells that become B cells.|
|B-cell prolymphocytic leukemia||A very rare and aggressive form of leukemia that develops in immature B cells.|
|Non-Hodgkin’s lymphoma||The |
|Hairy cell leukemia||A rare subtype of CLL that causes cancer cells with short, thin projections that look like hairs.|
|Splenic lymphoma with villous lymphocytes||A subtype of non-Hodgkin’s lymphoma that primarily involves the spleen and bone marrow.|
T-cell disorders include:
|Sezary syndrome||An aggressive form of a subtype of non-Hodgkin’s lymphoma called cutaneous T-cell lymphoma.|
|T-cell prolymphocytic leukemia||An extremely rare and aggressive cancer characterized by out-of-control growth of mature T-cells.|
|Adult T-cell leukemia-lymphoma||A rare and fast-growing type of non-Hodgkin’s lymphoma that affects people with human T-lymphotropic virus type 1.|
|Large granulated lymphocyte leukemia||A slow-growing type of CLL that starts in T cells.|
Natural kill cell disorders
Natural kill cell disorders include:
|Aggressive natural killer cell leukemia||A rare leukemia that develops in natural killer cells.|
|Chronic lymphoproliferative disorders of natural killer cells||A sustained increase in mature natural killer cells for longer than 6 months without a clear cause.|
Symptoms will vary depending on which type of disorder you are diagnosed with.
Many lymphoproliferative disorders can cause some combination of the following:
- sore throat
- swollen lymph glands
- spleen enlargement or liver enlargement
- elevated white blood cell counts
- abnormal bleeding or bruising
- excessive bleeding
- unintentional weight loss
- night sweats
- loss of appetite
- bone pain
- frequent infections
- nausea and vomiting
Lymphoproliferative disorders develop when the mechanisms that control your body’s normal growth of lymphocytes break down. Researchers have identified specific risk factors for some types of lymphoproliferative disorders, but often the underlying cause is not known.
The causes of some specific types of lymphoproliferative disorders include:
- X-linked lymphoproliferative disorder: This disorder is linked to a mutation in the X chromosome that predisposes people to natural killer cell and T-cell lymphoproliferative disorders. It causes the immune system to overreact to the Epstein-Barr virus, which is estimated to affect more than
90%of people worldwide.
- Autoimmune lymphoproliferative syndrome: This syndrome is caused by a mutation in the genes that control your lymphocytes. People with this syndrome have immune systems that remain active even without the presence of an infection. The most common gene affected is called FAS.
- Post-transplant lymphoproliferative disorder: This disorder is caused by immunosuppression after an organ transplant. It causes the uncontrolled replication of B cells.
Potential risk factors
Genetic changes, whether inherited or acquired, can cause some lymphoproliferative disorders. For example, most people with CLL have a change in a chromosome. The most common genetic change is the loss of part of
People with immunodeficiency are more likely to develop lymphoproliferative disorders. Some conditions linked to a
- common variable immunodeficiency
- Chediak–Higashi syndrome
- severe combined immunodeficiency
- Wiskott-Aldrich syndrome
- invasive fungal infections
- viral infections such as HIV
Undergoing an organ transplant or taking drugs that suppress your immune system (such as cyclosporin, sirolimus, and tacrolimus) may also increase your risk.
Doctors generally start the diagnostic process by considering your medical and family history and performing a physical exam.
You may receive blood tests to check for markers of some blood disorders or viruses such as cytomegalovirus or Epstein-Barr virus. Blood tests may also be used to look for genes associated with certain disorders.
Imaging can help search for tumors or other signs of disease. Scans may include:
Biopsies, or small tissue samples, are needed to confirm the diagnosis of many types of blood cancers. Depending on what type of cancer you have, you may receive a biopsy of:
Healthcare professionals will analyze the biopsied cells in a lab to look for signs of disease.
Multidrug chemotherapy is used to treat most types of blood cancer.
Most lymphoproliferative disorders associated with the Epstein-Barr virus
Autoimmune lymphoproliferative disorders are treated by targeting the underlying autoimmune disease. The first-line treatment often includes corticosteroids and immunoglobulin administered through an IV.
Post-transplant lymphoproliferative disorder often goes away once immunosuppressants are stopped, and sometimes with antiviral therapy.
The severity of lymphoproliferative disorders varies drastically between conditions. For example, diffuse large B-cell lymphoma has a 5-year relative survival rate of about
Factors that may influence your survival include:
- the type of lymphoproliferative disorder you have
- age — younger age is generally associated with better survival
- your overall health
- the extent of disease progression
- certain genetic factors
What is a relative survival rate?
A relative survival rate provides an estimate of how long someone with a specific condition may live after their diagnosis compared with someone without the condition.
For example, a person with a 5-year survival rate of 70% is 70% as likely to live for 5 years as someone without the condition.
Always remember that these figures are just estimates. It’s best to discuss your outlook with your doctor as they know more about your specific health condition.
Lymphoproliferative disorders are a large group of conditions that cause white blood cells called lymphocytes to replicate uncontrollably. These conditions can be cancerous or noncancerous.
In many cases, doctors do not know exactly what causes these disorders. Gene mutations usually play a role. Some mutations are inherited from your parents while others might be acquired throughout your life.
The outlook for lymphoproliferative disorders varies widely. Some disorders do not require any treatment while others may be life threatening.
Your doctor can give you the best idea about what to expect if you’re diagnosed with a particular lymphoproliferative disorder.