TREATMENT

There are two potentially life-threatening complications seen in children with NHL : superior vena cava syndrome and/or mediastinal tumor with airway obstruction most often seen in lymphoblastic lymphoma; and tumor lysis syndrome, most often seen in small noncleaved cell NHL.

Supportive care of children with NHL consists of :

• Complications arising from the space-occupying nature of the tumor
  
  
• Metabolic complications of chemotherapy (i.e. tumor lysis syndrome)
  
  

Management of Complications due to Space-Occupying Nature of the Tumor at Specific Anatomic Sites

Intrathoracic :Always maintain patient in semi-sitting or sitting-up position and avoid use of general anesthesia. Diagnostic procedures should include :

• Chest radiograph to evaluate :
  
  
  • Size of mass
    
    
  • Degree of airway compression
    
    
  • Presence of significant amounts of pleural and/or pericardial effusions
    
    
• Echocardiogram for evaluation of pericardial effusion and cardiac function.
  
  
• Biopsy of a clinically involved peripheral lymph node under local anesthesia or from aspiration of pleural effusion, when present. If the disease is present exclusively in the anterior mediastinum, attempt biopsy (under local anesthesia) through a small suprasternal incision, or if possible, obtain a fine needle biopsy.
  
  

It is possible that all of the aforementioned procedures may be prohibitive because of the patient's poor clinical condition. Under this circumstances, treat the patient with corticosteroid with or without a limited radiation field until the mass is sufficiently small to permit safe biopsy under general anesthesia. Corticosteroid and/or local radiotherapy can bring about rapid resolution of SVC obstruction.

Management of Pericardial Effusion

Pericardial effusion can cause life-threatening cardiac tamponade. Pericardial rub, S-T segment elevation on electrocardiogram (ECG), a globular-shaped heart on a chest radiograph, and cardiac ultrasonography establish the diagnosis. When signs of cardiac tamponade (pulsus paradoxus, elevated venous pressure, or hypertension) are present, pericardiocentesis should be performed. The cytology of the fluid should be examined. Prompt treatment with chemotherapy is necessary to prevent the reaccumulation of fluid.

Management of Gastrointestinal Complications

Abdominal disease is more commonly observed in patients with small noncleaved non-Hodgkin's lymphoma. The following complications are of immediate significance:

Small bowel obstruction: NHL of the GI tract commonly involves the terminal ileum and the cecum. The tumor can cause bowel obstruction, either by compression of the bowel lumen or by intussusception. In about 25% of patients, the tumor can be resected completely. Chemotherapy should begin within a few days of surgery.

Gastric bleeding or perforation: Endoscopic examination should be performed to identify patients at high risk for massive bleeding and/or perforation by noting the extent of involvement of the stomach wall, degree of ulceration, and necrosis. High-risk patients should be treated with resection of the tumor (involving total or partial gastrectomy, depending on the extent of the tumor) before starting chemotherapy.

Management of Tumor Lysis Syndrome

In patients with high tumor burden, especially those with stage III and stage IV small noncleaved cell lymphoma, B-cell acute lymphoblastic leukemia, and T-cell leukemia/lymphoma syndrome, the following metabolic complications can occur before starting chemotherapy:

• Renal failure
  
  
• Hyperuricemia
  
  
• Hyperphosphatemia
  
  
• Hypocalcemia
  
  
• Hyperkalemia
  
  

These complications are observed in the previously mentioned conditions because these tumors have high growth fractions and they are exclusively sensitive to chemotherapy. As a consequence, there is rapid release of intracellular metabolites (such as phosphorous, potassium, and uric acid) in quantities that exceed the excretory capacity of the kidneys.