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Leukemia and Lymphoma

As a healthcare provider, you participate in every aspect of care for patients facing a cancer diagnosis. Use this section to refresh your knowledge of leukemia and lymphoma or share the information with patients who are facing a leukemia or lymphoma diagnosis.

Facts and Statistics

According to the American Cancer Society:

  • An estimated 44,790 new cases of leukemia are expected in the US in 2009, with 21,870 deaths from the disease.
    • Approximately 5,760 new cases of acute lymphocytic leukemia (ALL) will be diagnosed in the US in 2009.
      • ALL is most prevalent in children between 2 and 4 years of age.
    • Approximately 12,810 new cases of acute myeloid leukemia (AML) will be diagnosed in the US in 2009.
      • AML is generally a disease of the elderly and is uncommon before the age of 40
    • Approximately 15,490 new cases of chronic lymphocytic leukemia (CLL) will be diagnosed in the US in 2009.
      • CLL accounts for one-third of all leukemias and primarily affects older adults.
    • Approximately 5,050 new cases of chronic myeloid leukemia (CML) will be diagnosed in the US in 2009.
      • CML accounts for 10% to 15% of all leukemias, with an average age of diagnosis of 67 years.
  • Non-Hodgkin's lymphoma (NHL) is one of the most common cancers in the US, with an estimated 65,980 new cases expected in 2009.
    • The average lifetime risk of developing NHL is 1 in 50.

Risk Factors

While some risk factors for leukemia and lymphoma are unavoidable, such as ethnicity or gender, other risk factors, such as environmental risk factors, are avoidable.

  • Ethnicity: Caucasians are diagnosed with leukemia more often than African-Americans, Hispanics, and Asians.
  • Gender: Men are diagnosed with leukemia more frequently than women.
  • Age: The risk of developing leukemia increases with age.
  • Infection: Two viral diseases have been linked to the development of leukemia, the Epstein-Barr virus and the human T-cell lymphoma/leukemia virus. Although not all people infected with these viruses go on to develop leukemia, there is a correlation. There also may be a link between infection with the human immunodeficiency virus (HIV) and leukemia.
  • Ionizing Radiation: Radiation s the best known and most studied risk factor for leukemia. CLL is one of the few leukemias believed not to be linked to radiation exposure.
  • Nonionizing Radiation: While there is no conclusive evidence, nonionizing radiation may be linked to the development of ALL.
  • Occupational Exposure: Exposure to certain chemicals, such as benzene, may increase the risk of leukemia. Similarly, those who work in petroleum manufacturing, chemical manufacturing, rubber manufacturing, and shoe manufacturing and those who work with printers and paints also may be at increased risk.
  • Antineoplastic Agents: Exposure to some chemotherapeutic agents, particularly those destructive to the bone marrow, may increase the risk of subsequent leukemia.
  • Smoking: Exposure to cigarette smoke may increase the risk of developing leukemia.
  • Immune Suppression: Immune suppression, including that brought about by medical treatment, may increase the risk of leukemia.
  • Chronic Immune Response: While the cause of NHL is not known, chronic immune response, typically elicited by infection with certain bacteria or viruses, is one of the few risk factors associated with the development of NHL.

Screening and Early Detection

There are no routine screening procedures for leukemia and lymphoma. It is typical for a patient to seek medical treatment when symptoms appear or for an "accidental" diagnosis to be made when a blood test is ordered for another reason.

Diagnosis and Staging

Diagnosing leukemia and lymphoma can be difficult and confusing. Several tests are available to assist in confirming the diagnosis, including blood tests, genetic testing, biopsy, and bone marrow aspiration.

Staging for each of the leukemias and lymphomas is different.

  • ALL is not staged in the same manner as solid tumors. Several systems have been used to classify ALL, one of the most common being the French-American-British (FAB) Classification.

    FAB Subtype Approximate % of Adult ALL Patients Immunologic Type Comments
    L1 30% T cell or pre-B cell
    L2 65% T cell or pre-B cell
    L3 5% B cell Poor prognosis with standard therapy. Also called Burkitt's leukemia.
  • AML, similar to ALL, is not staged in the same manner as solid tumors. Several systems have been used to classify AML, one of the most common being the French-American-British (FAB) Classification.

    Subtype Name % of Adult AML Patients Prognosis Compared to Average for AML
    M0 Undifferentiated acute myeloblastic leukemia 5% Worse
    M1 Acute myeloblastic leukemia with minimal maturation 15% Average
    M2 Acute myeloblastic leukemia with maturation 25% Better
    M3 Acute promyelocytic leukemia 10% Best
    M4 Acute myelomonocytic leukemia 20% Average
    M4 eos Acute myelomonocytic leukemia with eosinophilia 5% Better
    M5 Acute monocytic leukemia 10% Average
    M6 Acute erythroid leukemia 5% Worse
    M7 Acute megakaryoblastic leukemia 5% Worse
  • CLL typically is staged according to the Rai Staging System in the US.
    • Rai Stage 0: The blood lymphocyte count is >10,000 lymphocytes/mm3 of blood (lymphocytosis). The lymph nodes, spleen, and liver are not enlarged and red blood cell and platelet counts are near normal.
    • Rai Stage I: Lymphocytosis plus enlarged lymph nodes. The spleen and liver are not enlarged and red blood cell and platelet counts are near normal.
    • Rai Stage II: Lymphocytosis plus an enlarged spleen (and possibly an enlarged liver), with or without enlarged lymph nodes. Red blood cell and platelet counts are near normal.
    • Rai Stage III: Lymphocytosis plus anemia (too few red blood cells), with or without enlarged lymph nodes, spleen, or liver. Platelet counts are near normal.
    • Rai Stage IV: Lymphocytosis plus thrombocytopenia (too few blood platelets), with or without anemia or enlarged lymph nodes, spleen, or liver.
  • For practical purposes, the Rai stages may be separated into low-, intermediate-, and high-risk groups when determining treatment options.
    • Stage 0 is considered low risk.
    • Stages I and II are considered intermediate risk.
    • Stages III and IV are considered high risk.
  • CML is divided into 3 groups that help predict prognosis:
    • Chronic Phase: Characterized by <10% blasts in blood or bone marrow samples. These patients usually have fairly mild symptoms (if any) and usually respond to standard treatments. Most patients are diagnosed in the chronic phase.
    • Accelerated Phase: In this phase, the bone marrow or blood samples have >10% but <20% blasts. Symptoms may include fever, poor appetite, and weight loss. CML in the accelerated phase does not respond as well to treatment as CML in the chronic phase. The leukemia cells often have developed new chromosome changes, in addition to the Philadelphia chromosome.
    • Blast Phase (Acute Phase or Blast Crisis): Bone marrow and/or blood samples have >20% blasts. The blast cells often spread to tissues and organs beyond the bone marrow. These patients often have fever, poor appetite, and weight loss. At this point, CML acts much like an aggressive acute leukemia.
  • NHL may be staged according to the Ann Arbor Staging Classification:
    • Stage I: The cancer is located in a single region, usually 1 lymph node and the surrounding area. Stage I disease often will not have obvious outward symptoms.
    • Stage II: The cancer is located in 2 separate lymph node regions, and both areas are on the same side of the diaphragm – that is, both are above the diaphragm or both are below the diaphragm.
    • Stage III: The cancer involves lymph nodes or organs on both sides of the diaphragm.
    • Stage IV: Presence of either of the following indicates stage IV:
      • Cancer has spread to multiple spots of an organ (or multiple organs) outside the lymph system. Cancer cells may or may not be found in the lymph nodes near these organs.
      • Cancer has spread to only 1 organ outside the lymph system, but lymph nodes far away from that organ are involved.
  • In addition, the following letters can be added to the stages:
    • A: No symptoms
    • B: Presence of "B symptoms" (fever, night sweats, weight loss >10% of body weight)
    • E: Used if the disease is "extranodal" (not in the lymph nodes) or has spread from lymph nodes to adjacent tissue.
    • X: Used if the largest tumor is >10 cm large (also called "bulky disease"), or if the lymph node in the center of the chest (mediastinal node) is wider than one-third of the chest on a chest X-ray.

The leukemias are subdivided into acute or chronic forms, with each type of leukemia identified based on the level of maturation of the cells involved.

  • ALL: ALL is the most common form of childhood leukemia. ALL is characterized by extreme proliferation of lymphocyte cells in the bone marrow and in the lymphatic system. B cells or T cells may be involved.
  • AML: AML occurs in both adults and children, but it is far less common in young people. The cells involved in AML are the myeloblasts that proliferate uncontrollably and prevent other needed cells from functioning.
  • CLL: CLL is the most common adult leukemia and has been considered a disease of elderly men. However, increasing numbers of younger people also are being diagnosed with CLL, possibly because routine blood exams are more common and may be facilitating earlier detection. With CLL, affected lymphocytes divide slowly but in a poorly regulated manner. They live much longer than usual and over time are unable to perform their proper functions. CLL is further subclassified into 3 categories:
    • B-CLL (more than 95% of all CLL cases) also has been described as small lymphocytic lymphoma. In one instance, it behaves like a leukemia with a high white blood count, and in the other, it behaves like a small lymphocytic lymphoma, with tumorous lumps in the lymph nodes.
    • CLL/PL is a mixture of CLL cells and prolymphocytes in the blood. Ten percent to 55% of the cells are prolymphocytes.
    • CLL/Mixed is diagnosed when variations in cell sizes are seen, with some cells larger than CLL lymphocytes. Some have irregular nuclei that appear to have clefts in them.
  • CML: Characterized by the presence of the Philadelphia chromosome.

The non-Hodgkin's lymphomas are classified into several subgroups. There are multiple classification systems used to identify NHL subtypes. The following outlines the World Health Organization (WHO) classification system:

  • Diffuse Large B-cell Lymphoma: A more common form of NHL characterized by large cells when viewed under the microscope
  • Follicular Lymphoma: Characterized by cells that grow in a circular or nodular pattern within the lymph nodes
  • Small Lymphocytic Lymphoma (Chronic Lymphocytic Leukemia): Two closely related diseases that many consider to be variations of the same disease
  • Mantle Cell Lymphoma: A very rare form of lymphoma associated with small- to medium-sized cells
  • Marginal Zone B-cell Lymphoma: Also a rare form of lymphoma, associated with small cells
  • Primary Mediastinal B-cell Lymphoma: Accounting for 2% of all lymphomas, the cells are large and resemble diffuse large B-cell lymphomas
  • Burkitt's Lymphoma: Primarily affecting men, cells are medium sized
  • Lymphoplasmacytic Lymphoma (Waldenström's Macroglobulinemia): Accounting for only 1% to 2% of all lymphomas, cells are small and found mainly in the bone marrow, lymph nodes, and spleen
  • Hair Cell Leukemia: Characterized by small B lymphocytes with projections resembling "hairs," typically found in the bone marrow, spleen, and circulating blood
  • Primary Central Nervous System Lymphoma: May involve the brain, spinal cord, tissue around the spinal cord, and the eye
  • Precursor T-lymphoblastic Lymphoma/Leukemia: May be considered a leukemia or a lymphoma depending on the extent of bone marrow involvement. Cancer cells are typically small- to medium-sized immature T-cells
  • Peripheral T-cell Lymphomas: Comprises 7 sub-types of lymphomas that develop from more mature T-cells

Leukemia & Lymphoma Cancer Cancer Treatment Options

As a healthcare provider, you may find yourself educating patients about their treatment options. The following is a brief summary of common therapies used to treat leukemia and lymphoma.

  • ALL: Therapy for ALL is one of the most complex treatment plans used in any type of cancer. Treatment typically follows 3 phases: induction therapy, consolidation therapy, and maintenance therapy.
  • AML: AML has most commonly been treated with induction chemotherapy. If remission does not occur, induction chemotherapy can be repeated or a new chemotherapy regimen initiated. Once a remission is achieved, which is defined as <5% blast cells seen in the bone marrow, consolidation chemotherapy is typically given for 4 or 5 more cycles (4 to 6 months).
  • CLL: Potential treatments for CLL include chemotherapy, monoclonal antibodies, surgery (rarely), radiation therapy, and bone marrow or stem cell transplant.
  • CML: Potential treatments for CML include targeted therapies, interferon, chemotherapy, radiation, surgery (rarely), and bone marrow or stem cell transplant.
  • NHL: Chemotherapy, biologic therapy, radioimmunotherapy, and bone marrow or stem cell transplants are treatment options for NHL.

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