CHILD LYMPHOBLASTIC LYMPHOMA

Child Lymphoblastic Lymphoma is a type of non-Hodgkin lymphoma of the lymphatic system. Child lymphoblastic lymphoma is similar in etiology to acute lymphoblastic leukemia because lymphoblasts infiltrate nodal structures of the bone marrow, spleen, and central nervous system. A difference in this disease is that the tumor may present as a lump in some part of the body, with the most common location being in the abdomen. Occasionally, it may present as a lump in a lymph gland, bone, skin, thyroid gland, or the tonsils.

Diagnostic testing: Diagnostic testing for child lymphoblastic lymphoma may include:

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  Blood and urine tests;

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  Surgical biopsy;

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  Fine needle aspiration biopsy;

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  Bone marrow aspiration and biopsy; and

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  Imaging such as ultrasound, MRI, CT scan and PET scan.

Physical findings: Signs and symptoms of childhood lymphoblastic lymphoma may be different depending on the location of the tumors. Common symptoms may include:

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  High fever for no known cause;

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  Night sweats;

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  Weight loss of more than 10% of body weight in a short period of time (within 6 months);

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  Enlarged liver and kidney;

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  Generalized weakness;

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  Painless swelling of the lymph nodes in the neck, underarm, stomach or groin;

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  Difficulty breathing;

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  Wheezing; and

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  Coughing.

ICD-9: 200.8

The childhood form of lymphoblastic lymphoma is a disease of rapid onset and progression. In most cases, the disease has progressed to an advanced stage (stage III or IV) by the time of diagnosis. The prognosis depends on histology (structure of the affected tissue); extent of the disease; presence or absence of metastasis; the child’s age; and response to therapy.

Children with lymphoblastic lymphoma are treated with chemotherapy and radiation, usually following acute leukemia treatment protocols. Chemotherapy often includes intrathecal therapy (injection of chemotherapy into the spinal canal). Radioimmunotherapy is being researched as a possible treatment option.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes diagnostic features of the disorder and laboratory findings are needed to confirm the diagnosis;

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  Pediatric oncology consultation reports;

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  Imaging studies;

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  Biopsy reports; and

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  Pathology reports.

Suggested Listings for Evaluation:

REMARKS

Must meet listing level severity.