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- Feb 09 2017
Burkitt Lymphoma: Diagnosis, Prognosis, Symptoms, and Treatments
Signs and symptoms
Burkitt and Burkitt-like lymphomas have a rapid and aggressive clinical course with frequent bone marrow and central nervous system (CNS) involvement. These are considered to be medical emergencies and require immediate diagnostic and therapeutic intervention.
Endemic (African) Burkitt lymphoma (eBL) most commonly involves the jaw and facial bone (orbit) (>50% of cases). Sporadic Burkitt lymphoma (sBL) most often presents as abdominal tumors with bone marrow involvement. Immunodeficiency-related Burkitt lymphoma cases usually as with nodal involvement with frequent bone marrow involvement.
The National Cancer Institute staging system is as follows:
A - Single solitary extra-abdominal site
AR - Intra-abdominal, more than 90% of tumor resected
B - Multiple extra-abdominal tumors
C - Intra-abdominal tumor
D - Intra-abdominal plus 1 or more extra-abdominal sites
The Ann Arbor system and St. Jude/Murphy staging (commonly used) consist of 4 stages.
Stage I is as follows:
Single tumor (extranodal)
Single anatomic area (nodal)
Stage II is as follows:
Single tumor (extranodal) with regional node involvement
Primary gastrointestinal tumor
Lymphoma involving nodal areas on the same side of the diaphragm
Stage IIR consists of completely resected intra-abdominal disease.
Stage III is as follows:
Lymphoma involving sites on opposite sides of the diaphragm
All primary intrathoracic tumors
All paraspinal or epidural tumors
Extensive intra-abdominal disease
Stage IV consists of any of the above, with CNS or bone marrow involvement (< 25%) at presentation.
The risk-adapted approach is used to treat most patients in the present-day clinical setting. Patients are divided into 2 broad groups, low- and high-risk patients. Low-risk patients have nonbulky disease (< 10 cm), early stage (I or II) disease, good performance status, and a normal lactate dehydrogenase (LDH) level. High-risk patients include all other patients.
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