NON-HODGKIN'S LYMPHOMA

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Last Updated : 1/4/2011
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Bharat R Agarwal
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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.



Contributor Information and Disclosures Bharat R Agarwal
Pediatric Hematologist-Oncologist, Division of Pediatric Hem-Onco, B.J. Wadia Hospital for Children, Mumbai, India


First Created : 1/4/2001
References
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