Hairy Cell Leukemia Symptoms and Treatment Guidelines

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What is the life expectancy of someone with hairy cell leukemia? How can you prevent hairy cell leukemia? Can you die from hairy cell leukemia? Let’s find out about hairy cell leukemia symptoms and treatment!

Table of Contents

Hairy Cell Leukemia Definition

Hairy cell leukemia meaning – What is hairy cell leukemia? Hairy cell leukemia is a blood cancer that begins in the bone marrow.

Hairy Cell Leukemia Causes

What causes hairy cell leukemia? What is the most common cause of hairy cell leukemia?

Hairy cell leukemia is a rare type of cancer that starts in hematopoietic stem cells that have changed into mature B lymphocytes with hairy cytoplasmic projections.

The V600E mutation in the BRAF gene is known to cause hairy cell leukemia because it can be found in almost all cases when the disease is first found and when it comes back.

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Hairy Cell Leukemia Diagnosis

Pancytopenia.
Splenomegaly, usually a big spleen.
There are hairy cells in the blood and especially in the bone marrow.

Laboratory Findings

Pancytopenia is what makes hairy cell leukemia stand out. Anemia is almost always present; thrombocytopenia and neutropenia are present in 75% of patients. Most of the time, there aren’t many “hairy cells” on a peripheral blood smear. They look different because they have many cytoplasmic projections.

Most of the time, you can’t get any fluid out of the bone marrow (it’s a “dry tap”), and the diagnosis is made by looking at the shape of the bone marrow. When tartrate-resistant acid phosphatase is used to stain the hairy cells, they have a unique pattern (TRAP). Immunophenotyping shows that the cells simultaneously show the antigens CDllc, CD20, CD22, CD25, CD103, and CD123. When the spleen is looked at under a microscope, there are a lot of hairy cells in the red pulp. This is different from how lymphomas usually tend to affect the white pulp of the spleen.

Differential Diagnosis

Other lymphoproliferative diseases, like Waldenstrom macroglobulinemia and non-Hodgkin lymphomas, should be distinguished from hairy cell leukemia. It can also be confused with other causes of pancytopenia, such as hypersplenism from any cause, aplastic anemia, and paroxysmal nocturnal hemoglobinuria.

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Hairy Cell Leukemia Symptoms

Signs and symptoms of hairy cell leukemia – What are some symptoms of hairy cell leukemia?

Most of the time, middle-aged men get this disease. The average age at presentation is 55, and there are 5 times as many men as women.

Most patients get tired slowly, while others have symptoms related to a spleen much bigger than usual, and some come to the doctor because of an infection.

Splenomegaly happens almost always and can be very big. However, in 50% of cases, the liver is bigger, and lymphadenopathy is rare.

Most of the time, hairy cell leukemia is a slow-moving disease characterized by pancytopenia and repeated infections, including mycobacterial infections.

See also Waldenström Macroglobulinemia Symptoms and Treatment Guidelines

Hairy Cell Leukemia Treatment and Management Guidelines

Treatment of hairy cell leukemia – What is the best treatment for hairy cell leukemia?

Treatment is needed for diseases that cause symptoms, such as pain in the spleen, repeated infections, or significant cytopenias. The best treatment is a nucleoside analog, like pentostatin or cladribine, which is given as a single course and cures 70–95% of patients.

There are infectious side effects of treatment, so patients should be closely watched. However, the average response length is more than 8 years. Patients who relapse a year or more after their first treatment can be again given one of these drugs. Rituximab can be used alone or in combination with a nucleoside analog when a patient has relapsed.

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Patients with refractory or relapsed hairy cell leukemia have a 100% overall response rate to the BRAF inhibitor vemurafenib, with 35–40% going into complete remission. However, the average amount of time between relapses is 19 months for people who had a complete remission and 6 months for people who had a partial response.

Moxetumomab pasudotox is a recombinant immunotoxin that targets CD 22 and is approved for people with diseases that don’t respond to other treatments. The most important trial showed that 31% of patients got better and stayed better. But the diphtheria toxin part of it can cause blood vessels to leak and cause a condition called hemolytic-uremic syndrome.