CLINICAL FEATURES

The presenting symptoms of NHL depend mainly on the location of the tumor. It may present in a variety of ways, occasionally providing a major diagnostic dilemma because of the protean manifestations of its presentation.

Burkitt's lymphoma may be of the endemic or sporadic variety. Table 2 lists the epidemiologic, immunologic, and molecular features of these two varieties.

Table 2 Epidemiologic, Immunologic, and Molecular Features of Endemic and Sporadic Burkitt's Lymphoma

Features	Endemic	Sporadic
Epidemiologic Population affected	African	Worldwide
Age affected	Children(peak age, 7 years)	Young adults (peak age, 11 years)
Organ involvement	Jaw, paraspinal, ovary	Marrow, abdomen, nasopharynx, ovary
Epstein-Barr virus	Present in more than 97% of cases	Present in less than 30% of cases
CNS involvement	More frequent than bone marrow involvement	As frequent as bone marrow involvement
Immunologic IgM secretion	Little or none	Prominent
Fc and C3	+	-
CALLA	+-	-
IgH gene rearrangement	DH or JH	IgH switch region
Molecular Breakpoints on chromosome 8	Upstream of C-myc	Within C-myc
Site of cell origin	Germinal center of lymph node	Bone marrow

The frequency of involvement, according to primary sites and tumor distribution of NHL at the time of diagnosis, is as follows.

Abdomen: The primary site in 35% of cases is abdominal - the ileocecal region, appendix, ascending colon, or some combination of these sites. These patients usually present with:

• Abdominal pain
  
  
• Vomiting and diarrhea
  
  
• Abdominal distension
  
  
• Palpable mass
  
  
• Intussusception
  
  
• Peritonitis
  
  
• Ascites
  
  
• Acute gastrointestinal (GI) bleeding
  
  
• Obstructive jaundice
  
  
• Hepatosplenomegaly
  

Bleeding and perforation of the intestine occur infrequently in patients with Burkitt's lymphoma. Lymphoma is the most frequent anatomic lesion causing intussusception in children over 6 years of age. When this disease presents insidiously, it may clinically and radiologically resemble Crohn's disease.

Head and Neck: In 13% of cases, the head and neck are involved, causing enlargement of the cervical node(s) and parotid gland, jaw swelling, and unilateral tonsillar hypertrophy. The disease may present with nasal obstruction, rhinorrhea, hypoacousia, and cranial nerve palsies.

Mediastinum

The frequency of involvement of the mediastinum is 26%. A large anterior mediastinal mass may produce superior vena caval syndrome (distended neck veins, edema of the neck and face, marked dyspnea, orthopnea, dizziness, headache, dysphagia, epistaxis, altered mental status, and syncope associated with bending). In this condition, a large anterior mediastinal mass compresses the superior vena cava (SVC) because of thinness of its wall and its close apposition to the vertebral column. The rapid growth of the mass does not permit enough time to develop effective collateral circulation to compensate and results in signs and symptoms of SVC compression.

Tumors of the mediastinum have a marked tendency to involve the bone marrow, transform to acute lymphoblastic leukemia, and develop meningeal and gonadal involvement. May of these tumors are composed of cells of T-lymphocytes lineage. Pleural effusion may be produced by direct pleural involvement and/or may result from the compression of lymphatics by the mediastinal mass. The presence of pericardial effusion may cause cardiac tamponade.

Other Primary Sites

Other sites involved (11%) include skin and subcutaneous tissue, orbit, thyroid, bone (with or without hypercalcemia), kidney, epidural space, breast, and gonads. The peripheral nodes are affected in 14% of cases.

Table 3 Evaluation of Patient with Non-Hodgkin's Lymphoma

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• History and physical examination
• Blood count
• Serum electrolytes: blood urea nitrogen (BUN), creatinine, liver chemistries, calcium,phosphorus, magnesium
• Viral studies : HIV antibody, hepatitis A,B, C serology, cytomegalovirus (CMV) antibody,varicella antibody, herpes simplex virus (HSV) antibody
• Bone marrow aspiration and biopsy*
• Spinal fluid, peritoneal, pericardial, or pleural fluid examination - cytochemical, immunologic,cytogenetic, and molecular
• Adequate surgical biopsy for cytochemical, immunologic, cytogenetic, and molecular studies
• Evaluation of hepatic function (bilirubin, alkaline phosphatase, serum glutamic-oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), serum protein electrophoresis)
• Lactic dehydrogenase (LDH) levels
• Soluble interleukin-2 receptor (IL-2R) levels (if possible)
• Evaluation of renal function (urinalysis, BUN, uric acid, creatinine)
• Chest radiograph
• Computed tomography (CT) scan of the chest (when indicated)
• Ultrasonography of the abdomen
• CT Scan of the abdomen with contrast
• Plain radiograph and CT scan of afflicted site as deemed necessary on clinical grounds
• Gallium scan
• Diphosphonate bone scan - bone scan prior to gallium scan, because gallium will obscure the diphosphonate (if bone scan positive, radiographs or CT scan of bone or MRI of involved area indicated)
• Magnetic resonance imaging (MRI) especially for bone involvement (e.g. vertebrae)
• Dental evaluation in patients with Burkitt's lymphoma

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* May establish diagnosis without lymph node biopsy.

Note : Avoid undue delay in carrying out investigations because of risk of rapid dissemination of the disease, especially to the bone marrow. In urgent medical situations (e.g. cord compression, cranial nerve palsy, or renal failure), minimum investigations prompted by the patient's particular clinical problems should be carried out and patient should be treated on day of admission.