Burkitt Lymphoma and Burkitt-like Lymphoma Clinical Presentation
- Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD more...
History and Physical Examination
Burkitt lymphoma (BL) is a highly aggressive B-cell lymphoma. All the symptoms are caused by rapid turnover of the mature B lymphocytes and the involvement of extranodal sites and invasion of contiguous organs.
Because of the rapid growth of the Burkitt tumor, patients may quickly manifest significant metabolic derangement and renal function impairment. Less common presentations of Burkitt lymphoma (BL) include an epidural mass, skin nodules, central nervous system (CNS) symptoms, and bone marrow involvement.
Burkitt lymphoma (BL) can also present as acute lymphocytic leukemia (L3-ALL), with fever, anemia, bleeding, and adenopathy.
eBL, sBL, and immunodeficiency-associated BL
The 3 different clinical variants of Burkitt lymphoma (BL) described (endemic, sporadic, and immunodeficiency related) may have varied clinical presentations.[3, 7, 37]
The endemic form of Burkitt lymphoma (eBL) is most commonly seen in patients in equatorial Africa, with jaw and facial bone (orbit) involvement occurring in more than 50% of cases. Patients most often present with swelling of the affected jaw or other facial bones, loosening of the teeth, and swelling of the lymph nodes, which are nontender and rapidly growing, in the neck or below the jaw. Other clinical presentations including abdominal masses (ileal, cecal, etc), as well as ovarian, gonadal, skeletal, and breast involvement have also been documented.
The sporadic forms (sBL) most often present with abdominal tumors with bone marrow involvement. Patients most commonly present with abdominal tumors that cause swelling and pain in the affected area. Some patients present with symptoms of bowel obstruction secondary to an ileal-cecal intussusception caused by tumor growth. However, generalized lymphadenopathy is rare. Approximately 90% of patients with sBL and 50% of patients with eBL have abdominal masses upon presentation.
Involvement of the Waldeyer ring (palatine and lingual tonsils, nasopharyngeal adenoid) and nodes is common in sBL, and may result in dysphagia, dyspnea, wheezing, and airway obstruction. Jaw tumors are very rare in sBL.
Like eBL, sBL can involve the ovaries, kidneys, and breasts. Cases of Burkitt lymphoma presenting as acute pancreatitis, cardiac masses, bilateral ovarian masses, and even skin lesions have been reported.[39, 40, 41, 42]
Immunodeficiency-related Burkitt lymphoma usually presents as nodal involvement. Bone marrow involvement occurs frequently. Waldeyer ring and mediastinal nodes are rarely involved.
Patients with Burkitt-like lymphoma (BLL) may have a variable presentation compared with those with classic Burkitt lymphoma (BL). Patients with BLL usually present in their fourth or fifth decade of life. More than 50% of affected patients present with widespread (nodal and extranodal) disease with frequent bone marrow and peripheral blood involvement. Some patients have a leukemic presentation.
After reviewing Southwest Oncology Group (SWOG) data, Braziel et al reported the following clinical features of BLL : Median age was reported as 47 years (range, 28-69 y), and 70% of the patients presented with bulky advanced disease (stage II, III, or IV), with 60% of patients having two or more extranodal sites of lymphoma involvement.
Major signs of Burkitt lymphoma include a soft-tissue mass associated with the involvement of the jaw or other facial bones, rapidly enlarged cervical lymph nodes, abdominal masses, and ascites. The physical examination findings depend on the sites of extranodal involvement and may include the following:
Palpable tumor of the mandibulomaxillary region
Ecchymosis and/or petechiae (as a result of thrombocytopenia)
Meningeal signs (from CNS disease)
Summary of common clinical manifestations
Common findings and symptoms in patients with Burkitt lymphoma are summarized below.
Abdominal masses, which can cause abdominal pain and distention andascites
Nausea and vomiting
Loss of appetite, change in bowel habits, or both
Signs and symptoms of acute abdomen
Mandibular or maxillary mass
Most common presentation in eBL
Maxillary tumors are more common (may involve the orbit)
Jaw involvement occurs much less frequently (15-20% of sBL cases)
Meningeal infiltration, with or without cranial nerve (CN) involvement (frequently, CN III and CN VII); this is the most common mode of presentation with CNS disease
Headaches, visual impairment, and paraplegia from spinal involvement; these may be the initial presenting features in some cases
"B" systemic symptoms
B systemic symptoms are uncommon but may be associated with other presenting symptoms, such as fever, weight loss, night sweats, fatigue.
Bone marrow involvement is common in Burkitt lymphoma
Lymphadenopathy is more common in affected adults than in children
Patients with more than 25% bone marrow involvement are usually referred to as having Burkitt leukemia (BL). However, most patients will present with nodal or extranodal masses. Different staging systems, such as the National Cancer Institute (NCI) and Ann Arbor and St. Jude/Murphy staging systems, as well as a risk-adapted approach, have been proposed for these patients and are summarized below.
NCI staging system
See the list below:
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
Ann Arbor system and St. Jude/Murphy staging (commonly used)
Single tumor (extranodal)
Single anatomic area (nodal)
Single tumor (extranodal) with regional node involvement
Primary gastrointestinal tumor
Lymphoma involving nodal areas on the same side of the diaphragm
Completely resected intra-abdominal disease
Lymphoma involving sites on opposite sides of the diaphragm
All primary intrathoracic tumors
All paraspinal or epidural tumors
Extensive intra-abdominal disease
Any of the above, with central nervous system (CNS) or bone marrow involvement (< 25%) at presentation
Around 30% of patients present with limited-stage disease (stage I or II), and 70% present with more extensive disease (stage III or IV). Bone marrow involvement is found in 30-38% of patients, and the CNS is involved in 13-17% of adult patients.
Currently, most patients are treated following a risk-adapted approach. In this classification, patients are broadly divided into two groups: low risk and high risk.
Low-risk patients are those who 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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