Non-Hodgkin's Lymphoma, Part 1 — Introduction, Symptoms and Treatment

What is Non-Hodgkin's Lymphoma?

Non-Hodgkin's lymphoma is a malignant solid tumor of the immune system, and arises from undifferentiated lymphoid cells. Lymphoid cells normally can be found circulating throughout the body. For this reason, non-Hodgkin's lymphoma can arise anywhere, and should be considered a generalized disease from the outset. The disease often spreads in a pattern that follows the typical circulation of normal lymphoid cells. This includes lymph nodes and the spleen. However, the tumor cells can also spread in a random, diffuse, unpredictable and aggressive manner, involving tissues outside the lymphoid system, such as bone marrow and spinal fluid. The tumors grow very rapidly and create a large tumor burden, which is the amount of cancerous cells in proportion to the rest of the body. Non-Hodgkin's lymphoma can be divided into categories based on the predominant tissue types seen under the microscope, including Lymphoblastic, Undifferentiated and Large Cell.

Incidence and prevalence

Collectively, lymphomas are the third most common childhood malignancy, after acute leukemias and brain tumors. Lymphomas constitute between 10 and 12 percent of childhood cancers. In older adolescents, the incidence of non-Hodgkin's lymphoma increases and they become the second most common malignancy in that age group, surpassing brain tumors.

Location of Cancer Incidence
per
100,000
All Sites 15.9
Leukemias 3.8
Brain and other nervous tissue 2.8
Hodgkin's Lymphoma 1.3
Non-Hodgkin's Lymphoma 1.1
Bone and Joint 1.0
Soft Tissue 1.0
Kidney and Renal Pelvis 0.7

The incidence of non-Hodgkin's lymphoma (NHL) appears to be rising in the U.S. This trend is driven in large part by the occurrence of NHL in patients who are immunocompromised, such as children who are infected with HIV (human immunodeficiency virus) or are currently receiving chemotherapy or radiation for an unrelated cancer.

Symptoms

Presenting symptoms depend on the location of the largest or original tumor. In the abdomen, it might cause pain, distention or obstruction of the intestine, a palpable mass (one that can be felt), obstruction of the bile duct or bleeding in the stools. In the head and neck, it can present with enlarged lymph nodes in the neck, jaw swelling, tonsil enlargement, obstruction of the nose, or dysfunction of the cranial nerves. In the chest it might cause venous obstruction showing as distended neck veins and swelling of the head and neck, trouble breathing, fluid around the lungs or heart, or even heart failure. Bone marrow involvement will be seen through abnormal blood counts.

Staging of the tumor is determined by the number of tumors, the anatomic areas involved, whether the disease is on both sides of the diaphragm and whether the disease extends to the central nervous system (brain and spinal cord) or the bone marrow.

Treatment

Surgery is principally limited to a biopsy for diagnostic purposes only. Rarely, it is done to resect part of the tumor in emergency situations. Multiple-agent chemotherapy is the first choice of therapy for nearly all children with non-Hodgkin's lymphoma. The tumor responds to many different agents. Many children with non-Hodgkin's lymphoma receive chemotherapy intrathecally, or directly into the spinal fluid. The tissue type of the non-Hodgkin's lymphoma and its stage determine the exact chemotherapy treatment. Drugs include cyclophosphamide, vincristine, methotrexate, prednisone, L-asparaginase, cytarabine, daunorubicin, 6-mercaptopurine and thioguanine.


For more information

Part 2 — Possible Medication Side Effects, Restrictions, and Implications for School

http://www.cancer.gov

The Leukemia and Lymphoma Society

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White Plains, NY 10605
Phone: 914-949-5213
Fax: 914-949-6691
Non-Hodgkin's Lymphoma

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National Cancer Institute
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National Cancer Institute

Information Resources
Cancer Information Service (CIS)
1-800-4-Cancer (1-800-422-6237)
TTY 1-800-332-8615

Contributed by:

Robert Trueworthy, MD
Professor
Chief of Pediatric Hematology and Oncology
University of Kansas Medical Center

Lavonne Ridder, ARNP, CPON
Clinical Nurse Specialist
Pediatric Hematology and Oncology
University of Kansas Medical Center

Kathy Davis, MSEd, PhD
Associate Professor
Project Director, Connected Kansas Kids
Director, KU Kids Healing Place
University of Kansas Medical Center