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CCS Prep

Coding for Non-Hodgkin's Lymphoma

Among the new ICD-9-CM diagnosis codes that will be effective Oct. 1, six new codes representing Non-Hodgkin's Lymphoma (NHL) will be available for use. It's important that coders understand the clinical differences between the varieties of the disease in order to classify it appropriately. It's estimated that more than 63,000 new cases of NHL will be diagnosed in the U.S. in 2007, with approximately 18,660 deaths due to the disease.

Lymphoma is generally defined as a malignancy that begins in the lymph nodes, which are the building blocks of the body's immune system and play a significant role in fighting infection. Because lymph nodes are found throughout the body, lymphoma may involve various sites as well, although it's most commonly found in lymph nodes of the groin, neck or armpits. When white blood cells (lymphocytes) become abnormal and begin to subdivide uncontrollably, lymphoma may develop, which can then spread to almost any other part of the body via the lymphatic channels.

Hodgkin's Lymphoma is one type of lymphoma, but all others are generally grouped together as Non-Hodgkin's Lymphoma, which is much more common than Hodgkin's Lymphoma. These tumors are also classified according to the aggressiveness of their growth.

  • Indolent or low-grade lymphomas tend to grow slowly and typically cause few symptoms. Most NHLs are considered indolent and are treated with chemotherapy and/or radiation therapy.
  • Aggressive or intermediate-grade lymphomas grow and spread more quickly and may cause severe symptoms.
  • Very aggressive or high-grade lymphomas grow very rapidly and account for a smaller percentage of total cases. Unless treated quickly, these lymphomas may be life threatening.

    Over time, many indolent lymphomas become more aggressive, thus changing their designation from indolent to aggressive or very aggressive. Although the precise cause of NHL is unknown, there are several generally accepted risk factors for developing the disease.

    While young people may develop NHL, the risk increases with age; most patients with NHL are more than 60 years of age. Patients with a weak immune system from an inherited condition, Human Immunodeficiency (HIV) disease or certain drugs are also at increased risk. Finally, it is thought that having certain types of infections may increase the risk of developing NHL. These infections include Epstein-Barr virus (EBV), Helicobacter pylori (H. pylori), Human T-cell leukemia/lymphoma virus (HTLV-1), Hepatitis C virus or the HIV virus. Researchers are also currently investigating possible links between herbicide or other chemical exposure and the development of NHL.

    When a patient presents with indolent NHL without symptoms, treatment may consist of close monitoring or "watchful waiting" to ensure that treatment begins as soon as symptoms appear. Treatment of NHL also depends on the staging, or determination of how many areas of the body are affected. The stages are as follows:

  • Stage I: the lymphoma cells are found only in a single lymph node group (such as in the axilla or neck).
  • Stage II: the lymphoma cells are in at least two lymph node groups on the same side (either above or below) the diaphragm.
  • Stage III: the lymphoma is in groups of lymph nodes both above and below the diaphragm or may also be in an organ or tissue near these lymph node groups.
  • Stage IV: the lymphoma is throughout at least one organ or tissue in addition to the lymph nodes, or it is in the blood, liver or bone marrow.

    Treatment of aggressive or very aggressive NHL primarily consists of chemotherapy, radiation therapy or biological therapy. Biological therapy for NHL typically involves infusion of monoclonal antibodies, which are lab-produced proteins that bind to the malignant cells and help the patient's own immune system kill them. For patients with disease recurrence or relapse, stem cell transplantation may be required.

    To classify various types of NHL more specifically, new codes have been developed for lymphoma tumors that have previously been classified to code 202.8 [Other lymphomas]. The new codes include:

  • Marginal Zone Lymphoma?new code 200.3: This type of tumor is typically indolent and accounts for approximately 10 percent of all lymphomas.

    Subcategories that may be documented in the medical record include: Extranodal marginal zone B-cell lymphoma, Mucosa associated lymphoid tissue (MALT), Nodal marginal zone B-cell lymphoma and Splenic marginal zone B-cell lymphoma.

  • Mantle Cell Lymphoma?new code 200.4: Mantle cell is an aggressive tumor type and represents about 6 percent of all lymphomas. It is typically considered "incurable" with traditional treatment but stem cell transplantation may be provided, depending on the patient characteristics and presentation.
  • Primary Central Nervous System (CNS) Lymphoma?new code 200.5: Primary CNS lymphoma is also an aggressive tumor but only accounts for 1 percent to 2 percent of all lymphomas.

    This type of tumor requires different chemotherapy options than other NHLs and in many cases also requires radiation to the brain. Younger patients may have brain dysfunction, while older patients may present with NHL-related dementia.

  • Anaplastic Large Cell Lymphoma (ALCL)?new code 200.6: this tumor type is considered aggressive and accounts for 2 percent of all lymphomas. Patients are tested for ALK-1, which is a fusion protein. If the patient is positive for the protein, (ALCL ALK-1+), there is usually a correlation to a younger age and better prognosis.
  • Peripheral T-cell Lymphoma?new code 202.7: Peripheral T-cell NHL is also classified as aggressive and usually occurs in approximately 7 percent of all lymphomas. This tumor type carries the worse prognosis of all lymphomas and typically is associated with extranodal (tumor involving organs in addition to lymph nodes) presentation.
  • Large Cell Lymphoma?new code 200.7: this tumor type is considered very aggressive, grows extremely quickly and accounts for approximately 30 percent of all lymphomas.

    Because the new NHL codes are found in categories 200 and 202, fifth digits are required for their assignment. The coder must carefully review the medical record documentation to determine the site of NHL presentation. The corresponding fifth digits include:

    0 unspecified site, extranodal and solid organ sites

    1 lymph nodes of head, face and neck

    2 intrathoracic lymph nodes

    3 intra-abdominal lymph nodes

    4 lymph nodes of axilla and upper limb

    5 lymph nodes of inguinal region and lower limb

    6 intrapelvic lymph nodes

    7 spleen

    8 lymph nodes of multiple sites

    Coders should also ensure that the physician has documented actual lymphoma before assigning these codes. A patient may have a secondary malignancy or metastasis to a lymph node, which is not classified as lymphoma.

    Metastatic lymph node tumors are classified to category 196 (Secondary and unspecified malignant neoplasm of lymph node, with the appropriate fourth digit for the specific site affected. Refer to Coding Clinic, 2nd Quarter 1992, pp. 3-4 for a discussion of lymph node primary and secondary tumors. After review of this Coding Clinic issue, along with the ICD-9-CM Official Coding Guidelines related to neoplasms, test yourself with the following quiz, utilizing the new codes above:

    1. A patient with previously diagnosed large cell lymphoma presents with intractable bone pain due to lymphomatous spread to the bone. The appropriate diagnosis code(s) is/are:

    a. 198.5, 200.70

    b. 200.70

    c. 170.9

    d. 170.9, 200.70

    2. A 62-year-old patient is seen for biopsy of masses in both the chest and the groin lymph nodes. Pathology reports reveal nodal marginal zone B-cell lymphoma. The appropriate diagnosis code(s) is/are:

    a. 196.1, 196.5

    b. 200.32, 200.35

    c. 200.38

    d. 200.82, 200.85

    3. A patient presents to the hospital radiology department for a work-up of left lower quadrant abdominal pain. He has Peripheral T-cell lymphoma of the axilla, in remission. At the beginning of the encounter (before any radiology findings are available), the appropriate diagnosis code(s) is/are:

    a. 789.00, 202.70

    b. 789.04, V10.79

    c. 789.04, 202.74

    d. 202.74, 789.04

    This month's column has been prepared by Cheryl D'Amato, RHIT, CCS, director of HIM, and Melinda Stegman, MBA, CCS, clinical technical editor, Ingenix (, which specializes in the development and use of software and e-commerce solutions for managing coding, reimbursement, compliance and denial management in the health care marketplace.

    Coding Clinic is published quarterly by the American Hospital Association.

    CPT is a registered trademark of the American Medical Association.


    Answers to CCS PREP!: 1. b. When lymphoma involves the bone, it is still considered the primary tumor and should be classified to the lymphoma code only. Codes 198.5 and 170.9 are inappropriate for this type of bone tumor. Only code 200.70 should be assigned; 2. c. According to Coding Clinic, 2nd Quarter 1992, pp. 3-4: "If lymph nodes in more than one region of the body (e.g., head and thorax, neck and abdomen, axilla and lower limb and pelvis) are involved, the fifth digit "8" should be assigned." Code 200.38 is the correct code because it represents Marginal zone lymphoma of multiple sites; 3. c. The reason for the encounter is the abdominal pain; the most specific 789.04 code should be sequenced first because we have no radiology findings at this time. According to Coding Clinic, 2nd Quarter 1992, pp. 3-4: "Lymphoma patients who are in remission are still considered to have lymphoma and should be assigned the appropriate codes from categories 200-202." Code 202.74 should be assigned to represent Peripheral T-cell lymphoma of the axillary region lymph nodes.

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