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Hairy cell leukemia


Disease: Hairy cell leukemia Hairy cell leukemia
Category: Blood diseases & tumors

Disease Definition:

In hairy cell leukemia, the bone marrow makes too many B cells (lymphocytes), which is a type of white blood cells that fights infection. Hairy cell leukemia is a rare, slow-growing cancer of blood. The excess B cells are abnormal, and due to fine projections (villi) from their surface, they look "hairy" under the microscope. Fewer healthy white blood cells, red blood cells and platelets are produced once the number of leukemia cells increases.


Children and teenagers do not get hairy cell leukemia. It most commonly occurs in middle-aged or older adults and affects men more than women.


This disease has no cure, and its exact cause is still not known. Treating this disease could lead to a remission for years, however, because it could never completely disappear, hairy cell leukemia is considered a chronic disease.

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Symptoms, Causes


In some people, a blood test for another disease or condition could reveal hairy cell leukemia because they may never experience any signs or symptoms of this disease.


Other people may experience some of these signs and symptoms which are common to a number of diseases and conditions:


  • Fatigue
  • Weight loss
  • Easy bruising
  • Recurring infections
  • Weakness
  • A feeling of fullness in the abdomen which could make it uncomfortable to eat more than a little at a time.


A defect in the DNA causes cancer. In hairy cell leukemia, the DNA mutations cause the bone marrow stem cells to create too many white blood cells that don't work properly. However, the cause of the DNA mutations that lead to hairy cell leukemia is still not known.



Usually, this type of leukemia progresses very slowly, and in some cases it remains stable for years. That is why this disease has only a few complications. However, when a progressing hairy cell leukemia is left untreated, it could cause these serious complications:


Because the numbers of the white blood cells are low, the patient will be at risk of infections that their body would otherwise fight off.


When fewer cells are available to carry oxygen throughout the body because of low red blood cell count, anemia occurs, which causes fatigue.


Once a person starts bleeding, low platelet counts make it hard for them to stop bleeding. A person may notice that they bruise more easily in case they have a mildly low platelet count. Spontaneous bleeding from the nose or gums could be due to very low platelet counts.

Ruptured spleen:

When hairy cell leukemia cells fill the spleen, they cause the spleen to enlarge. The extra cells will eventually cause the spleen to burst. Although very rare, splenic rupture is a life-threatening condition that requires emergency surgery to remove the spleen.



Some studies have found that people with hairy cell leukemia might have an increased risk of developing a second type of cancer. Whether this risk is due to hairy cell leukemia's effect on the body, or if the risk comes from the medications that are used to treat this condition is still not clear.


Some of the common cancers that people with hairy cell leukemia later develop include thyroid cancer, Hodgkin's disease and non-Hodgkin's lymphoma.


People with hairy cell leukemia don't always require treatment. Some people prefer to wait to treat their cancer only if it causes signs and symptoms because hairy cell leukemia usually progresses very slowly and sometimes doesn't progress at all.
However, most of the people with hairy cell leukemia will need treatment at one point in their lives.


In case a person has been diagnosed with hairy cell leukemia, early treatment doesn't have any advantages even though they may be eager to rid their body of cancer. Hairy cell leukemia is treatable at all stages, unlike some other types of cancer, which means that remission won't happen less likely if someone waits before treating the cancer.


A person may decide to undergo treatment if their hairy cell leukemia causes signs and symptoms. Although new treatments are effective at putting hairy cell leukemia in remission for years, however, there's no cure for this disease.



The first line of treatment for hairy cell leukemia is considered chemotherapy. Through the use of chemotherapy, most people experience complete or partial remission. In treating hairy cell leukemia, two chemotherapy drugs are used:


Treatment regimens for hairy cell leukemia usually begin with cladribine. Over seven days, the patient will receive a continuous infusion of the drug intravenously. Most people who receive cladribine experience a complete remission that lasts for several years. People could be treated with cladribine again in case their hairy cell leukemia returns. Infection and fever are some of the side effects of cladribine.


This drug is given on a different schedule but still causes remission rates that are similar to cladribine. People who are being treated with pentostatin receive infusions every other week for three to six months. Nausea and vomiting, sensitivity to light, infection, fever and eye inflammation (keratoconjunctivitis) are some of the side effects that are caused by this medication.


A few people who have hairy cell leukemia won't achieve remission using chemotherapy drugs because they are resistant to them. There are other people who can't tolerate chemotherapy. For example, because these drugs suppress the immune system and make small infections much worse, people with infections must avoid chemotherapy.



Immunotherapy, which is a biological therapy, tries to make cancer cells more recognizable to the immune system, so that the immune system could set about destroying the cancer once it identifies the them. For treating hairy cell leukemia, two types of biological treatments are used. They are:


Even though rituximab, which is a monoclonal antibody, is used to treat non-Hodgkin's lymphoma, however, it can also be used to treat hairy cell leukemia. Rituximab may be considered in case chemotherapy drugs haven't worked, or if the patient cannot take chemotherapy drugs. Bleeding, headache, infection and fatigue are some of the side effects of this drug.


In case chemotherapy was not effective, or if the patient couldn't chemotherapy, they may receive alpha-interferon. People who are treated with alpha-interferon, which lasts over a year, usually experience partial remission. Flu-like symptoms, such as fatigue and fever are some of its side effects.



The first treatment that was used in hairy cell leukemia was splenectomy, which is surgery to remove the spleen. However, it is only used in some rare cases these days. In case someone's spleen has ruptured, or if it's enlarged and causing pain, splenectomy may be recommended. Removing the spleen usually restores normal blood counts, but it can't cure hairy cell leukemia. This is why splenectomy could be useful in people with uncontrollable infections. Just like all surgeries, splenectomy carries a risk of infection and bleeding. When someone's spleen is removed, it could end up causing inflammation of the blood vessels (vasculitis) and make the person susceptible to infection.


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