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Lymphoblastic lymphoma (LL) is a type of blood cancer that is related to leukemia, myeloma, and myeloproliferative neoplasms. Lymphoma cells are cancer cells that originate from lymphocytes, a type of white blood cell. Lymphoma occurs in the lymphatic system, part of the immune system that includes the lymph nodes, spleen, tonsils, and other organs that help remove bacteria, waste, and excess fluid from tissue and fight infections and cancers.
Lymphoblastic lymphoma is a rare type of non-Hodgkin lymphoma (NHL) that produces lymphoblasts (immature lymphocytes). Most cases of LL are T-cell lymphoma (cancer cells come from T lymphocytes), but it can also appear as a B-cell lymphoma. Lymphoblastic lymphoma is closely related to acute lymphoblastic leukemia (ALL) (also called acute lymphocytic leukemia), and the two are treated similarly.
Lymphoblastic lymphomas such as ALL usually affect young children and teens, but they also occur in adults over 50. LL typically begins in the thymus (an organ in the chest) or lymph nodes and spreads to other parts of the body including the spleen, liver, blood, bone marrow, skin, bone, and testicles.
The cause of lymphoma and other cancers is gene mutations, or changes to your DNA, that result in cells growing out of control. Certain general factors can increase your risk of developing lymphoma, including exposure to certain chemicals or ionizing radiation, inherited genes, certain infections, and a compromised immune system.
Lymphoblastic lymphoma has two distinct subtypes based on the type of lymphoblasts that have become cancer cells, either T cells or B cells.
T-cell lymphoblastic lymphoma (T-LL) — also called precursor T-cell lymphoblastic lymphoma — arises from lymphoblasts that normally become T cells. T-LL is the most common type of lymphoblastic lymphoma. T-LL is usually aggressive (fast-growing) and often begins in the thymus. The thymus is in the mediastinum, the central part of the chest where the heart and other organs sit in between the lungs, behind the sternum (breastbone).
B-cell lymphoblastic lymphoma (B-LL) — also known as precursor B-cell lymphoblastic lymphoma — arises from lymphoblasts that normally become B cells. B-LL is less common than T-LL and is generally less aggressive. B-LL that relapses (returns after treatment) frequently affects the central nervous system (CNS), which includes the brain and spinal cord.
Acute lymphoblastic leukemia primarily affects the blood and bone marrow, whereas lymphoblastic lymphoma primarily involves the lymph nodes. Lymphoblastic lymphoma can also involve the blood and bone marrow. LL becomes ALL when more than 20 percent to 25 percent of a person’s bone marrow cells are lymphoblasts. Both LL and ALL have T-cell and B-cell subtypes: LL is most commonly the T-cell subtype, but ALL is most commonly the B-cell subtype.
Lymphoblastic lymphoma symptoms can vary based on the subtype of LL and the location of tumors. LL shares symptoms with many other types of non-Hodgkin lymphoma, including:
T-cell lymphoblastic lymphoma usually begins in the thymus and causes swelling of lymph nodes in the mediastinum. Swelling and inflammation in the mediastinum can put pressure on organs in the chest, causing symptoms such as:
These symptoms can be severe and require emergency treatment.
B-cell lymphoblastic lymphoma usually begins in lymph nodes and spreads to other lymphoid organs and throughout the body, including the skin and bones. When B-LL recurs after treatment, it frequently involves the brain and spinal cord.
Diagnosis of lymphoblastic lymphoma begins with a thorough medical history and physical exam to identify which symptoms and physical signs of disease you have, as well as your risk factors. Additional tests used to diagnose and stage LL include:
Accurate diagnosing and staging helps your doctor decide on the best treatment plan and determine disease prognosis (outlook).
Imaging tests help find tumors throughout the body, valuable in determining the cancer stage. Types of imaging tests used include X-rays, ultrasound, CT scans, positron emission tomography scans, and MRI.
Several blood tests are used to help diagnose lymphoblastic lymphoma. These tests include a complete blood count, tests for lactate dehydrogenase (LDH) levels, standard tests for liver and kidney function, and tests for diseases that affect the immune system, including HIV and viral hepatitis.
Biopsies entail collecting tumor cell samples to identify the type of cancer. Depending on the size and location of suspected cancer cells, a biopsy may involve surgery to remove all or part of a tumor (surgical or excisional biopsy), or it may involve taking a sample of a tumor using a needle (core needle biopsy and fine needle aspiration). Tumors that are deep inside the body are usually biopsied using one of the types of needle biopsy. Bone marrow biopsy (removing a small portion of bone and bone marrow) is also important for diagnosing lymphoma or leukemia. Lymphoblastic lymphoma may be diagnosed with a lymph node biopsy.
Cells taken from biopsies and blood samples are also typically studied in a lab to look for genes and proteins that can help identify specific types of cancer. Immunophenotyping identifies types of surface antigens (proteins on the cell surface) using flow cytometry or immunohistochemistry. Other techniques, such as in situ hybridization, are used to look for specific genetic mutations in the cancer cells. Surface antigens and genetic mutations are important for identifying the type of cancer and determining which treatments will work best.
Cancer staging helps determine the outlook for lymphomas. Most types of lymphoma are staged using the Lugano classification system. Stages are based on whether the lymphoma is localized or widespread, especially if the lymphoma has spread from one side of the diaphragm to the other. Stages are defined further by the size, or bulk, of the tumor and whether or not lymphoma has spread beyond the lymphatic system.
Lymphoma at stage 1 or 2 is considered early- or limited-stage. The lymphoma will involve lymph nodes, one organ outside the lymphatic system on the same side of the diaphragm, or both. Lymphoma at stage 3 or 4 is late- or advanced-stage. In these stages, the lymphoma has spread to both sides of the diaphragm and may include organs outside of the lymphatic system
Doctors also use the International Prognostic Index (IPI) to assess the outlook of lymphomas. The IPI uses five factors to classify a person’s risk of a poor outcome:
Treatment of lymphoblastic lymphoma involves a combination of chemotherapy, radiation, targeted therapy, and stem cell transplant. Specific treatment regimens are selected based on a person’s age and overall health, the subtype of their LL, and specific markers found in cancer cells.
Treatment for LL involves long-term chemotherapy over the course of two to three years, typically in three phases: induction, consolidation (intensification), and maintenance. A wide range of chemotherapy drugs are used to treat LL and ALL. According to the American Cancer Society, some of the most commonly used chemotherapy drugs for lymphoblastic lymphoma and ALL include:
Radiation therapy is not a standard treatment for lymphoblastic lymphoma, but it can be used in certain situations. Radiation is used in combination with high-dose chemotherapy before stem cell transplantation. Radiation can also be part of CNS prophylaxis to prevent the cancer from spreading to the brain and spinal cord. Sometimes radiation is used to help treat specific symptoms, such as those stemming from large tumors in the mediastinum or bone pain caused by tumors.
Different kinds of lymphoblastic lymphoma respond to different targeted therapies. When LL cells have a specific mutation, called the Philadelphia chromosome, targeted therapy with tyrosine kinase inhibitors (TKIs) are used as part of treatment. TKIs include Gleevec (imatinib), Iclusig (ponatinib), or Sprycel (dasatinib).
Targeted immunotherapy treats cancer using monoclonal antibody drugs that bind to specific cell surface antigens. B-cell lymphoblastic lymphoma can be treated with Blincyto (blinatumomab), which attaches to proteins found on B cells and healthy T cells to cause an immune response against the cancer cells. Another B-cell surface protein is targeted by Besponsa (inotuzumab ozogamicin). This is a monoclonal antibody drug attached to a chemotherapy drug, enabling the chemotherapy to be taken directly to cancer cells.
Chimeric antigen receptor T-cell therapy, which engineers a person’s T cells to attack cancer, can also be useful for treating LL.
Stem cell transplantation is frequently used along with chemotherapy to restore normal bone marrow function and prevent relapse. Autologous (self) stem cell transplants or allogeneic (donor) stem cell transplants are used to replace bone marrow killed off during chemotherapy. This approach allows for higher doses of chemotherapy, which are more effective. Stem cell transplant can decrease the incidence of relapse after initial treatment.
Prophylactic (preventative) treatment is important to prevent spread to the central nervous system. Relapsed LL frequently appears in the CNS. CNS prophylaxis can include radiation to the brain and spinal cord and intrathecal chemotherapy. Intrathecal chemotherapy involves injecting drugs into the fluid that surrounds the spinal cord and brain.
Some people with LL or ALL enroll in clinical trials as a treatment option. Clinical trials test the effectiveness of new treatments and novel combinations of various chemotherapy and targeted drugs.
The five-year survival for combined adult and pediatric (childhood) lymphoblastic lymphoma is 69.9 percent.
Childhood LL has a much better outlook than adult LL with five-year survival rates of 90 percent for early-stage LL and over 80 percent for advanced-stage LL.
Adult LL survival rates vary by subtype. T-cell LL has a five-year survival rate of 40 percent to 63 percent and the less-common B-cell subtype has a five-year survival rate of 51 percent to 69 percent.
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Mark Levin, M.D.
is a hematology and oncology specialist with over 37 years of experience in internal medicine. Review provided by VeriMed Healthcare Network.
Learn more about him here.
Kristopher Bunting, M.D.
studied chemistry and life sciences at the U.S. Military Academy, West Point, and received his doctor of medicine degree from Tulane University.
Learn more about him here.
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