Non-Hodgkin Lymphoma; Diffuse Large B-cell Lymphoma; B-cell Lymphoma; T-cell Lymphoma; Peripheral T-cell Lymphoma; Follicular Lymphoma; Burkitt Lymphoma; Anaplastic Large Cell Lymphoma
Cancer cells that start in the lymphatic systems are called lymphomas. When cancer cells get into the lymphatic system, the cancerous cells can also spread to other organs and tissues in the body. Child Lymphoma is the third most common cancer in children. Most childhood lymphomas can be classified as one of the following four types:
Burkitt lymphoma (BL), or small noncleaved cell lymphoma (SNCCL)
Lymphoblastic lymphoma (LL) - adjudicators are reminded that lymphoblastic lymphoma is a type of lymphoma that is similar to acute leukemia and needs to be evaluated under the medical listings for 13.06 Leukemia
Diffuse large B-cell lymphoma (DLBCL)
Anaplastic large cell lymphoma (ALDL)
Signs of childhood lymphoma may include breathing problems and swollen lymph nodes. These children may also exhibit symptoms of breathing difficulties; wheezing; coughing; swelling of the head, neck, upper body or arms; experience difficulties swallowing, have painless swelling of the lymph nodes in the neck, underarm, stomach or groin; experience unexplained weight loss, night sweats, and fever.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Diagnostic testing for child lymphoma includes physical examination, blood and urine tests, lymph node biopsy, bone marrow aspirate and biopsy, lumbar puncture, removal of fluid from chest or abdomen for testing, X-rays, ultrasound, radionuclide bone scan, MRI, CT, and PET scan.
Physical findings: Childhood lymphoma may cause many different signs and symptoms, depending on the location of the tumors. Lymphoma that grows close to the surface of the body (sides of the neck, underarm area above the collarbone or in the groin area) may have enlarged nodes that are seen or felt as in lumps under the skin. Lymphoma that is in the abdomen area will cause it to become swollen and tender. Lymphoma that starts in the thymus gland or lymph nodes of the chest or near the windpipe (trachea) may cause swelling and a bluish-red skin color.
ICD-9: 200.2 (Burkitt lymphoma); 200.5 (Primary CNS lymphoma); 200.6 (Anaplastic large cell lymphoma); 200.7 (Large cell lymphoma); 202.0 (Follicular or lymphocytic lymphoma); 202.7 (Peripheral T-cell lymphoma); 202.8 (Malignant lymphoma NOS)
ONSET AND PROGRESSION
Lymphoma is usually a disease of rapid onset and progression. Although the prognosis greatly depends on histology, extent of the disease, presence or absence of metastasis; the child’s age; and response to therapy, the majority of children with newly diagnosed child lymphoma are considered to have an excellent prognosis. Children with recurrent lymphoma have a less favorable prognosis and require longer treatment. Children with lymphoma that involves the brain, spinal cord, bone marrow, liver or lung are also associated with a less favorable prognosis. If the child does not respond to chemotherapy drugs, the disease can cause rapid death.
By the time that a child is diagnosed with recurrent lymphoma, the lymphoma has spread to other parts of the body. Most children with recurrent lymphoma are treated with chemotherapy. Chemotherapy is the most important treatment for children with lymphoma because chemotherapy can reach all parts of the body and kill lymphoma cells wherever they may be. It is common to use a combination of drugs and treatment, including intrathecal therapy (injection of chemotherapy into the spinal fluid), that may last a number of months or years.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation: The adjudicator needs medical evidence from treating sources and hospitals. It should include clinical examination, imaging tests, biopsies, pathology reports, surgical procedures, pertinent treatment records, and up-to-date progress notes.
Suggested Listings for Evaluation:
Primary lymphoma (excluding lymphoblastic) which involves brain, spinal cord, bone marrow, liver, or lung meets listing 113.05A1. Recurrent or persistent lymphoma regardless of location, following initial treatment meets listing 113.05A2.
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