Understanding Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma

There were approximately 17-18 million new cancer cases diagnosed worldwide in 2018, of which about 7% – or 1.2 million people – were blood cancers.^1,2* While tremendous progress in the treatment of these cancers has been made and survival rates for common blood cancers have consistently improved over time, there is still work to be done.³ Certain blood cancers, like acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL), can be more difficult to treat because they are rare and have a more aggressive nature.^4,5,6,7,8

What Are ALL and LBL?

Acute Lymphoblastic Leukemia, sometimes called acute lymphocytic leukemia (ALL), is a type of cancer of the blood and bone marrow that affects lymphocytes, a type of white blood cell.⁹ ALL is the most common type of cancer in children, though it can also occur in adults.¹⁰ ALL develops in the bone marrow (where blood cells are made) when unhealthy lymphocytes grow out of control and impair bone marrow function.¹⁰ Lymphoblasts, a form of immature lymphocyte, accumulate in the blood and bone marrow, are unable to function properly, and eventually interfere with the production of healthy blood cells.¹⁰

Symptoms of ALL may include fatigue, bone pain, fever, dizziness, shortness of breath, bruising, pale skin, frequent or heavy bleeding and infections that do not subside or keep recurring.¹⁰ These symptoms can seem similar to the flu, so if a person is experiencing these symptoms and they persist longer than the flu typically would, it’s important to see a doctor.¹⁰ The doctor will begin with a blood test, called a complete blood count (CBC).¹¹ This test may detect abnormalities of blood cell production, such as anemia, or may identify leukemia cells directly. If abnormalities are found on CBC, an examination of the bone marrow is used to confirm the diagnosis of ALL.¹²

Lymphoblastic lymphoma (LBL) is a rare, fast-growing, aggressive subtype of Non-Hodgkin’s lymphoma (NHL), most often seen in teenagers and young adults.¹³ LBL is a cancer of immature lymphoblasts that arises more commonly in T cells than B cells, which are forms of lymphocytes.¹⁴ It is a very aggressive lymphoma – also called high-grade lymphoma – which means the lymphoma grows quickly with early spread to different parts of the body.^12,15

Symptoms of LBL include painless swelling in the neck, armpit or groin, shortness of breath, coughing and chest pain.¹⁶ Lymph nodes in the mediastinum, the central part of the chest between the lungs, are the most commonly affected.¹³ LBL is diagnosed through a lymph node biopsy and reviewing the cells under a microscope.¹⁷

How Are ALL and LBL Treated?

Although ALL and LBL are different types of blood cancer, they are very similar in appearance and behavior.¹² Both cancers are due to fast-growing lymphoblasts and both require intensive treatment to kill cancer cells.^4,5,6,7,8 Current treatment options used to treat ALL and LBL are geared towards killing cancer cells while allowing healthy cells to live.¹⁸ These treatments work by interfering with the production of proteins that cancer cells need to grow and divide.¹⁹

In some cases, treatment can prompt a strong immune response which manifests as hypersensitivity.²⁰ Approximately 30% of patients develop this allergy or immunity to treatment.²⁰ Common symptoms associated with clinical hypersensitivity include urticaria, also known as hives, dizziness and respiratory problems.²⁰ In some cases, potentially life-threatening anaphylaxis may occur.²⁰

Fortunately, the treatment landscape for cancers like ALL and LBL is ever-evolving and Jazz is committed to playing an important role in this evolution through the development of new therapies and educating healthcare providers, caregivers and patients, in particular when it comes to rare and difficult-to-treat cancers like ALL and LBL.

*Calculation based on data from the World Health Organization’s International Agency for Research on Cancer.

¹International Agency for Research on Cancer. Leukaemia. Available at http://gco.iarc.fr/today/data/factsheets/cancers/36-Leukaemia-fact-sheet.pdf. Accessed March 26, 2020.
²World Cancer Research Fund International. Worldwide Cancer Data. Available at https://www.wcrf.org/dietandcancer/cancer-trends/worldwide-cancer-data. Accessed March 27, 2020.
³Leukemia & Lymphoma Society. Facts and Statistics. Available at https://www.lls.org/facts-and-statistics/facts-and-statistics-overview/facts-and-statistics. Accessed March 27, 2020.
⁴Cancer Treatment Centers of America. Blood Cancers. Available at https://www.cancercenter.com/blood-cancers. Accessed March 20, 2020.
⁵American Cancer Society. Typical Treatment of Acute Lymphocytic Leukemia (ALL). Available at https://www.cancer.org/cancer/acute-lymphocytic-leukemia/treating/typical-treatment.html. March 20, 2020.
⁶American Cancer Society. Treatment of Children With Acute Lymphocytic Leukemia (ALL). Available at https://www.cancer.org/cancer/leukemia-in-children/treating/children-with-all.html. March 20, 2020.
⁷American Cancer Society. Treatment of Non-Hodgkin Lymphoma in Children, by Type and Stage. Available at https://www.cancer.org/cancer/childhood-non-hodgkin-lymphoma/treating/by-stage.html. Accessed March 20, 2020.
⁸Ryu IH, et al. Long-Term Survival after T-cell Lymphoblastic Lymphoma Treated with One Cycle of Hyper-CVAD Regimen. Cancer Res Treat. 2015;47(1):115–119.
⁹American Cancer Society. What Is Acute Lymphocytic Leukemia (ALL)? Available at https://www.cancer.org/cancer/acute-lymphocytic-leukemia/about/what-is-all.html. Accessed on March 26, 2020.
¹⁰Mayo Clinic. Acute Lymphocytic Leukemia Overview. Available at https://www.mayoclinic.org/diseases-conditions/acute-lymphocytic-leukemia/symptoms-causes/syc-20369077. Accessed March 26, 2020.
¹¹Mayo Clinic. Complete Blood Count (CBC). Available at https://www.mayoclinic.org/tests-procedures/complete-blood-count/about/pac-20384919. Accessed April 7, 2020.
¹²Mayo Clinic. Acute Lymphocytic Leukemia Diagnosis. Available at https://www.mayoclinic.org/diseases-conditions/acute-lymphocytic-leukemia/diagnosis-treatment/drc-20369083. Accessed March 26, 2020.
¹³Leukemia Foundation. Lymphoblastic Lymphoma. Available at https://www.leukaemia.org.au/disease-information/lymphomas/non-hodgkin-lymphoma/other-non-hodgkin-lymphomas/lymphoblastic-lymphoma/. Accessed March 18, 2020.
¹⁴Dana Farber Cancer Institute/Boston Children’s Cancer and Blood Disorders Center. Lymphoblastic Lymphoma. Available at http://www.danafarberbostonchildrens.org/conditions/leukemia-and-lymphoma/lymphoblastic-lymphoma.aspx. Accessed March 26, 2020.
¹⁵Cancer Research UK. High-Grade NHL. Available at https://www.cancerresearchuk.org/about-cancer/non-hodgkin-lymphoma/types/high-grade. Accessed March 26, 2020.
¹⁶MacMillan Cancer Support. Lymphoblastic Lymphoma. Available at https://www.macmillan.org.uk/cancer-information-and-support/lymphoma/lymphoblastic-lymphoma. Accessed March 19, 2020.
¹⁷Mayo Clinic. Lymphoma Diagnosis. Available at https://www.mayoclinic.org/diseases-conditions/lymphoma/diagnosis-treatment/drc-20352642. Accessed March 26, 2020.
¹⁸National Cancer Institute. NCI Dictionary of Cancer Terms. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms. Accessed March 18, 2020.
¹⁹Van der Sluis, I. M., et al. Haematologica. 2016;101(3): 279–285.
²⁰Zheng W, et al. Chin J Cancer Res. 2017;29(1):66–74.