T-cell prolymphocytic leukemia (T-PLL) is an aggressive type of leukemia affecting T lymphocytes (T cells), a white blood cell that helps the body fight infection.

In individuals with T-PLL, mutations in T-cell genes disrupt typical cell maturation, leading to uncontrolled growth and division. This causes cancerous cells to multiply rapidly and spread throughout the body, crowding out healthy cells.

This rare form of leukemia accounts for approximately 2% of adult mature lymphocytic leukemias. It most often affects people older than 65 years, although it can affect individuals in their 30s to 90s.

Read on to learn more about T-cell prolymphocytic leukemia, its symptoms, diagnosis, and treatment.

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T-PLL is a rare and aggressive type of blood cancer that affects T cells, a type of white blood cell. These cells are part of the immune system that helps fight infection.

In people with T-PLL, changes or mutations in T-cell genes disrupt typical cell maturation and cause the cells to grow uncontrollably and rapidly. The number of immature, nonfunctional T cells increase, making it difficult for the body to produce new, healthy blood cells.

As a result of these cellular changes, a person develops various signs and symptoms. Common signs of T-PLL include:

  • swollen lymph nodes
  • enlarged liver and spleen
  • night sweats
  • weight loss
  • fatigue
  • weakness
  • skin lesions or rashes
  • high white blood cell counts
  • low red blood cell counts, or anemia
  • low platelet counts

Doctors do not know the exact cause of T-PLL. However, it appears to involve certain mutations in T-cell genes that trigger typical T-cells to become leukemia cells.

The most common DNA changes are in proto-oncogene TCL-1, a cell growth and division gene. The genetic mutations turn it into an oncogene, which can trigger the growth of cancer cells.

Certain people, such as males and those over 65 years, are more likely to develop T-PLL. However, unlike other cancers, no known environmental risk factors exist.

T-PLL is aggressive, and the outlook is generally unfavorable.

The overall survival rate for T-PLL varies depending on several factors, including the individual’s age, general health status, and the extent of the genetic abnormalities.

The survival time for individuals with T-PLL is typically less than 2 years from diagnosis, although some individuals may survive longer with appropriate treatment.

Diagnosing T-PLL is complicated and typically involves several steps to confirm the presence of the disease and determine its extent. The initial evaluation often includes a physical examination and blood tests.

These tests assess the levels of blood cells, including T lymphocytes, and look for signs of anemia or other abnormalities.

Doctors may use the following tests to diagnose PLL:

  • Bone marrow biopsy: This checks if the bone marrow is healthy or making atypical cells.
  • Peripheral blood smears: These check for T-cell changes.
  • Immunophenotyping: This looks for markers on the surface of leukemia cells.
  • Chromosome analysis: This checks to see if there are genetic changes.
  • Imaging studies: These assess the extent of the disease.

T-PLL treatment involves a combination of therapies to control and manage the disease’s symptoms. The specific treatment approach depends on several factors, including the individual’s age, overall health status, and the extent of the disease.

Current treatment approaches include:

  • Watching and waiting: Around 10–15% of those with T-PLL do not have symptoms during diagnosis. Therefore, doctors may delay treatment and monitor the individual to check for disease progression.
  • Medications: For people with symptomatic T-PLL, drugs, including intravenous alemtuzumab (Campath) and alemtuzumab plus pentostatin (Nipent), may help control the disease.
  • Hematopoietic stem cell transplantation: Doctors may suggest a stem cell transplant in people who achieve disease remission following drug therapy. This is the only treatment that may offer a potential cure.

People with T-PLL should explore all available treatment options, including participating in clinical trials. These offer access to new and innovative therapies that can help improve outcomes for individuals with this disease.

Clinical trials may involve testing new drugs, drug combinations, or dosing schedules, allowing people to benefit from new therapies that may not yet be widely available.

T-PLL is an aggressive cancer that requires timely diagnosis and treatment.

If someone experiences symptoms relating to T-PLL, they should contact their doctor immediately for evaluation. Early diagnosis and treatment can help improve outcomes for those with the disease.

T-cell prolymphocytic leukemia (T-PLL) is a rare, aggressive form of blood cancer affecting white blood cells called T lymphocytes. When T cells become cancerous, they divide rapidly and atypically, crowding out healthy cells and spreading throughout the body.

A person then experiences T-PLL signs, such as fatigue, night sweats, and unexplained weight loss.

While the prognosis for T-PLL is generally unfavorable, treatment options are available to help manage the disease and improve outcomes.