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Medical Disability Advisor  >  Non Hodgkins Lymphoma

Non-Hodgkin's Lymphoma


Related Terms


  • Burkitt's Lymphoma
  • Diffuse Large Cell Lymphoma
  • Diffuse Small Cleaved Cell Lymphoma
  • Follicular Mixed Small Cleaved and Large Cell Lymphoma
  • Follicular Predominantly Large Cell Lymphoma
  • Follicular Small Cleaved Cell Lymphoma
  • Large Cell Immunoblastic Lymphoma
  • Lymphoblastic Lymphoma
  • Lymphoblastoma
  • Small and Large Cell Lymphoma
  • Small Lymphocytic Lymphoma
  • Small Noncleaved Cell Lymphoma

Differential Diagnoses


Specialists


  • Clinical Psychologist
  • General Surgeon
  • Hematologist
  • Oncologist
  • Pain Medicine Physician
  • Pathologist
  • Psychiatrist
  • Radiology Oncologist

Comorbid Conditions


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Factors Influencing Duration


Individuals older than 50 years tend to have more advanced disease and do less well in response to combination chemotherapy and radiation therapy than do younger individuals. The length of disability depends upon the stage of the disease when first detected, methods and complexity of treatment, and the individual's response to treatment. Disability may result from the adverse effects of radiation or chemotherapy, not only from the disease itself. Heavy physical labor is usually restricted for weeks to months following surgery, chemotherapy, and/or radiation therapy treatments.

Medical Codes


ICD-9-CM:
200 - Lymphosarcoma and Reticulosarcoma, Other Specified Malignant Tumors of Lymphatic Tissue
200.2 - Burkitts Tumor or Lymphoma; Malignant Lymphoma Burkitts Type
200.8 - Lymphoma, Other Named Variants; Lymphoma (Malignant): Lymphoplasmacytoid Type, Mixed Lymphocytic-histiocytic (Diffuse); Lymphosarcoma, Mixed Cell Type (Diffuse); Reticulolymphosarcoma (Diffuse)
202 - Neoplasms of Lymphoid and Histiocytic Tissue, Malignant
202.0 - Nodular Lymphoma; Brill-Symmers Disease; Follicular (Giant); Lymphocytic, Nodular; Follicular (Giant); Nodular; Reticulosarcoma, Follicular or Nodular
202.8 - Non-Hodgkins Lymphoma
202.9 - Malignant Neoplasms of Lymphoid and Histiocytic Tissue, Other and Unspecified; Follicular Dendritic Cell Sarcoma; Interdigitating Dendritic Cell Sarcoma; Langerhans Cell Sarcoma; Malignant Neoplasm of Bone Marrow NOS

Definition


Non-Hodgkin's lymphoma (NHL) is cancer that occurs in lymphoid tissue within the lymphatic system. The lymphatic system comprises important components of the body's immune system, including the lymph nodes, spleen, thymus gland, and tonsils.

The glands and vessels in the lymphatic system network circulate a clear, plasma-like fluid (lymph) throughout the body. Certain types of white blood cells (leukocytes) are manufactured by the lymphatic system to fight disease. Tissue cells in any organ system normally divide and reproduce in an orderly way. In NHL, certain cell types (B or T-lymphocytes) within the lymphatic tissue divide rapidly and replicate themselves uncontrollably, creating lesions or growths that destroy the normal structure of lymph system vessels. NHL tends to spread (metastasize) quickly and invade other organs such as the spleen, liver, and bones. Some types of NHL metastasize to unusual areas such as the central nervous system and the digestive (gastrointestinal) tract.

Cancers classified as NHL are sub-categorized into 1 of 15 or more different types of growths (malignant neoplasms) that may occur within the lymph system. The distinctions between these different categories of NHL are identified by the type of cells that produce the cancer, their rate of growth, and how invasive (metastatic) they are. They may be classified as high-grade (fast-growing or highly invasive), intermediate-grade (moderate growth and invasiveness), or low-grade (slow-growing or indolent).

Viruses have been shown to be associated with the development of NHL. Some association has been shown between the Epstein-Barr virus (EBV) responsible for infectious mononucleosis and the development of NHL, and also of Hodgkin's lymphoma. EBV is frequently found in tumors taken from individuals with non-Hodgkin's lymphoma and individuals who test positive for EBV show a two- to threefold increased risk of developing NHL. The human immunodeficiency virus (HIV), also appears to increase the risk of NHL, likely due to HIV-associated immunosuppression. The human T-cell lymphotropic virus type I (HTLV-1) has been shown to be linked to the development of a type of NHL called adult T-cell lymphoma (ATL).

Risk: Risk factors for NHL include occupational risks such as anesthesiology, carpentry, chemical industries, construction, engineering, farming, fishing, forestry, leather work, mechanics, metal working, road transport industry, rubber industry, sales and clerical work, and certain food industry occupation. Most of these associations are weak or inconsistent; further studies are underway to confirm these findings. Other risk factors may include exposure to vinyl chloride, pesticides, and hair dyes, as well as ingestion of certain prescription medications.

Incidence and Prevalence: The worldwide incidence of NHL ranges from 3.7 to 14.0 per 100,000 individuals, higher in more developed countries. The US incidence rate for NHL is 16 per 100,000 people for both men and women combined; the rate of development of new cases has been increasing since the 1970s, e.g., from 45,000 new cases in 1994 to 56,200 reported in 2001 (Nayak). The increasing incidence is generally attributed to the increase in HIV cases; NHL occurs in 15% of AIDS cases (Nayak). Generally NHL occurs in individuals older than 24 years of age, although the incidence increases with age, peaking after age 60.

Source: Medical Disability Advisor



History


History: Individuals usually report a painless or slightly tender, swollen lymph node in the neck, underarm, or inner thigh (ilioinguinal). Other symptoms may include fatigue, weight loss, unexplained fever, and sometimes night sweats. Gastrointestinal involvement can cause the individual to experience abdominal cramping or bloody diarrhea. Obstruction of the ureter that carries urine from the kidney may produce pain in the flank area (left or right side of the back near the lower ribcage). A history of virus infection (such as infectious mononucleosis or HIV), or Helicobacter pylori infection, may be reported.

Physical exam: Enlarged lymph nodes, nearly always in the neck region or armpit but occasionally in the groin, are usually noted during physical examination. Enlargement of both the spleen (splenomegaly) and liver (hepatomegaly) may be noticed during palpation of the abdomen. Tonsils may be enlarged as well. Bone tenderness or skin lesions may be noted.

Tests: Blood tests will typically be done, including a complete blood count (CBC) to determine red blood cell (RBC) count, white blood cell (WBC) count, hemoglobin level and platelet count. RBCs and hemoglobin may be reduced if anemia is present. WBCs may be elevated as part of the immune system's response to disease or inflammation. A stained differential blood smear may reveal abnormal white blood cells. Blood chemistries such as blood protein (serum albumin), serum protein electrophoresis, and the measurement of liver enzymes will be performed. An erythrocyte sedimentation rate (ESR) may be done to determine if inflammation is present; an ESR is typically elevated in NHL.

Definitive diagnosis of NHL requires a tissue biopsy of an enlarged lymph node; biopsies of other sites that could be involved may be done at the same time or after imaging studies identify specific sites. A section of the tiny piece of tissue removed by needle biopsy will be stained (immunohistochemical staining) and then examined microscopically by a pathologist who will identify cell types; the pathologist will stage the disease at the same time by classifying cells from different sites. The four possible stages for NHL are: I. A single node region or single extranodal site (outside lymph tissue); II. Two or more nodal regions and one extranodal site; III. Nodal disease on both sides of diaphragm, one extranodal site or spleen involvement; IV. Liver, bone marrow, or multiple extranodal sites. Flow cytometry studies may also be performed in addition to routine pathologic examination of cells. Other tests may include routine x-rays (radiographic examination) of the chest, liver, and spleen, and possibly bones, to identify lymphatic tissue that may be involved. Chest x-ray may reveal a mass in the middle of the chest (mediastinal mass). More detailed contrast-enhanced x-ray imaging of the lymph glands and lymphatic vessels (lymphangiography) may be done to determine the extent of lymph node involvement. Computer-aided x-ray analysis (computerized tomography), low-frequency radio waves (magnetic resonance imaging, or MRI), or an exploratory abdominal surgical procedure (laparotomy) may help determine if the cancer is spreading outside the lymph system to other organs. Positron emission tomography (PET) or gallium-67 scintigraphy may be performed to increase the accuracy of staging the cancer. These techniques use radioisotopes to enhance the visualization of biological differences in tumor tissue, allowing more detailed examination than other imaging methods provide. A sample of bone and bone marrow (bone marrow biopsy) may reveal the extent of bone involvement. Ultrasound imaging of the liver may reveal enlargement.

Source: Medical Disability Advisor



Treatment


Treatment will vary depending upon the type of NHL, the extent of the disease, and the organ systems involved in metastasis. Low grade types (including small cell lymphocytic, follicular small- cleaved cell, and follicular mixed small-cleaved and large-cell lymphomas) are usually treated with single-agent chemotherapy or combinations of chemotherapeutic agents. Administration of interferon alfa in conjunction with the chemotherapy has been shown to improve survival with up to 12 years disease-free remission (Smally 1118). Treatment may alternatively include immunotherapy with monoclonal antibodies, targeting substances on the surface of lymphoma cells but not on normal cells; this treatment is showing promise, with response rates as high as 70% (Nayak). Additionally, the antibody delivery of radioactive treatment (antibody-delivered therapy) has shown promising results; in one study, 84% of individuals with low-grade NHL showed complete remission. Intermediate grade NHL (including follicular predominantly large-cell, diffuse small cleaved-cell, diffuse small- and large- cell, and diffuse large-cell lymphomas) are systemic diseases when diagnosed and combination chemotherapy is the mainstay of treatment.

Novel therapeutic approaches include removal of a portion of the affected individual's bone marrow and storing it while chemotherapy or radiation treatment are administered to destroy cancerous cells. Later, the stored bone marrow is injected back into the individual (autologous bone marrow transplantation or autologous BMT) and becomes part of the individual's bone marrow (engraftment) with the objective of its return to normal function.

High grade NHL (including immunoblastic large cell lymphoma, lymphoblastic lymphoma, and small noncleaved-cell lymphomas such as Burkitt's lymphoma) have been treated most commonly with a combination of multi-drug chemotherapy and radiation treatments; autologous BMT has also been used.

Source: Medical Disability Advisor



Prognosis


Prognosis in adults varies widely and depends upon the type of NHL, the extent of the disease including whether it has just been diagnosed or has recurred, the organs involved in metastasis, the age and general condition of the individual, and the initial response to treatment. Individuals with low-grade lymphomas have a median survival rate of 5 to 10 years, those with intermediate-grade lymphomas have a median survival rate of 2 to 5 years, and individuals with high-grade lymphomas have a predicted median survival rate of less than 2 years (Estrada). In advanced stages, the disease is not usually curable. In general, the overall survival rate is 50% to 60% at 5 years and those with aggressive NHL have a 30% cure rate; most recurrences of the disease occur within the first 2 years after treatment; however, if the histology of the lymphoma remains low grade it can be re-treated with considerable success (Gajra).

Source: Medical Disability Advisor



Complications


Complications of non-Hodgkin's lymphoma may involve clinical emergencies such as lymph node enlargement or fluid accumulation in various locations in the body where obstruction may result, including: obstruction of the airway, obstruction of the major vein that returns blood to the heart (superior vena caval obstruction), compression of the heart (pericardial tamponade), spinal cord compression, obstruction of the hepatic (liver) bile duct (extrahepatic biliary obstruction), or pressure on nerves in the head or periphery (cranial and peripheral neuropathies). Complications of radiation treatment for NHL are similar to those of radiation for Hodgkin's lymphoma, including development of acute nonlymphocytic leukemia, autoimmune hemolytic anemia, and radiation-induced carcinomas and sarcomas. Additionally, radiation therapy to the neck region may result in an underactive thyroid (hypothyroidism) several years after treatment is completed. Chemotherapy often results in acute, though reversible, toxicity leading to nausea, vomiting, and neurologic disorders. Infections may occur as a result of immunosuppression by chemotherapeutic agents being used in treatment or as a result of the disease itself. Other complications of chemotherapy may include heart or lung disorders and female or male infertility.

Source: Medical Disability Advisor



Return to Work (Restrictions / Accommodations)


Individuals with non-Hodgkin's lymphoma of any type will experience high levels of fatigue with normal levels of physical exertion. Chemotherapy and radiation therapy can cause additional weakness and fatigue. Work responsibilities may need to be modified and heavy physical labor curtailed until recovery is complete.

Source: Medical Disability Advisor



Failure to Recover


If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case.

Regarding diagnosis:

  • Does individual have a history of Epstein-Barr virus (EBV) or human immunodeficiency virus (HIV)?
  • Does individual have human T-cell lymphotropic virus type I (HTLV-1)?
  • Does individual report a painless or slightly tender, swollen lymph node in the neck or inner thigh (ilioinguinal) region?
  • Has individual experienced unexplained fever and/or night sweats?
  • Does individual report abdominal cramping or bloody diarrhea?
  • Does individual note pain on the right or left side of the mid-back (flank)?
  • Was a sample of tissue (biopsy) from an enlarged lymph node taken to make a definitive diagnosis?
  • Were other tests performed, such as x-rays of the chest, liver, and spleen as well as computed tomography (CT scan), magnetic resonance imaging (MRI), or PET with radioisotopes?
  • Was abdominal exploratory surgery (laparotomy) performed to determine the spread (metastasis) of the disease?
  • Was a bone marrow biopsy obtained to reveal the extent of bone and bone marrow involvement, or a lymphangiography done to determine the extent of lymph node involvement?
  • Were blood tests such as a complete blood count (CBC), serum albumin, protein electrophoresis, and liver enzymes (serum lactate dehydrogenase) performed?
  • Was the diagnosis of non-Hodgkin's lymphoma confirmed? Is it high-, intermediate-, or low-grade NHL?
  • Has the disease spread (metastasized) into other organ systems?

Regarding treatment:

  • What is the type of NHL and extent of the disease?
  • If low-grade NHL, was one (single-agent) or more than one (combination) chemotherapeutic agent used?
  • Did individual receive radioactive antibodies (antibody-delivered therapy)?
  • If intermediate grade NHL, was combination chemotherapy used?
  • If high grade NHL, did the individual receive multi-drug chemotherapy and radiation treatments?
  • Were these treatments successful?
  • If not, is individual a candidate for autologous bone marrow transplantation?

Regarding prognosis:

  • What is the type of NHL, extent of the disease, and age of the individual?
  • Is this an initial diagnosis or a recurrence?
  • Has individual experienced complications from the disease, such as lymph node enlargement that compresses or obstructs vital organs and/or nerves?
  • Has individual experienced complications associated with radiation therapy, such as radiation-induced secondary cancer or hypothyroidism?
  • Has toxicity from chemotherapy treatments occurred resulting in nausea, vomiting, neurologic disorders, and/or suppressing the immune system?
  • What is the treatment plan for the complication and what is the expected outcome after treatment?
  • Has individual experienced grossly enlarged lymph nodes that alter appearance? Would individual benefit from psychological counseling to cope with body image changes and the impact of the disease?

Source: Medical Disability Advisor



Cited References


Estrada, Dolores A., Lakshmi Rajdev, and Joseph A. Sparano. "Lymphoma, Non-Hodgkin." eMedicine. Eds. Kashik A. Shastri, et al. 24 Jun. 2004. Medscape. 17 May 2005 <http://emedicine.com>.

Gajra, Ajeet, Neerja Vajpayee, and Sara Grethlein. "Lymphoma, B-Cell." eMedicine. Eds. Michael Paul Kosty, et al. 10 Jan. 2005. Medscape. 17 May 2005 <http://emedicine.com>.

Nayak, L., and D. Deschler. "Lymphomas." Otolaryngologic Clinics of North America 36 4 (2003): 625-646.

Smally, R., et al. "Adding Interferon Alfa to Induction Chemotherapy Improves Disease-Free Survival in Non-Hodgkin’s Lymphoma." Evidence-based Oncology 3 2 (2002): 1118-1122.

Source: Medical Disability Advisor






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