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Burkitt Lymphoma and Burkitt-like Lymphoma Clinical Presentation

  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Apr 08, 2016
 

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.[6]

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.[38] 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.

Burkitt-like lymphoma

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[43] : 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.

Signs

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:

  • Abdominal tenderness
  • Ascites
  • Abdominal mass
  • Hepatosplenomegaly
  • Palpable tumor of the mandibulomaxillary region
  • Ecchymosis and/or petechiae (as a result of thrombocytopenia)
  • Meningeal signs (from CNS disease)
  • Painless lymphadenopathy

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

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)

CNS involvement

  • 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.

Other

  • Bone marrow involvement is common in Burkitt lymphoma
  • Lymphadenopathy is more common in affected adults than in children
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Staging

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)

Stage I

  • Single tumor (extranodal)
  • Single anatomic area (nodal)

Stage II

  • Single tumor (extranodal) with regional node involvement
  • Primary gastrointestinal tumor
  • Lymphoma involving nodal areas on the same side of the diaphragm

Stage IIR

  • Completely resected intra-abdominal disease

Stage III

  • Lymphoma involving sites on opposite sides of the diaphragm
  • All primary intrathoracic tumors
  • All paraspinal or epidural tumors
  • Extensive intra-abdominal disease

Stage IV

  • 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.

Risk-adapted approach

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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Contributor Information and Disclosures
Author

Ali H Kanbar, MD Medical Oncologist/Hematologist, Dayton Cancer Center

Ali H Kanbar, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada, American Clinical and Climatological Association, International Society of Blood Transfusion

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,,Grifols<br/>Received honoraria from all the above companies for speaking and teaching.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

Patturajah Anbumani, MD, MBBS, MS, MCh Associate Medical Director, Best Medical Care; Former Associate Medical Director, Jeanes Hospital, Temple University Health System; Former Adjunct Clinical Assistant Professor, New York College of Osteopathic Medicine; Former Clinical Assistant Professor, Department of Medicine, State University of New York-Downstate

Patturajah Anbumani, MD, MBBS, MS, MCh is a member of the following medical societies: American College of Physicians, American Medical Association, and American Medical Women’s Association

Disclosure: Nothing to disclose.

Samer A Bleibel, MD Staff Physician, Department of Internal Medicine, Wayne State University School of Medicine, St John's Hospital and Medical Centers

Samer A Bleibel, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Asher A Chanan-Khan, MD Assistant Professor, Department of Medicine, Division of Lymphoma and Bone Marrow Transplantation, Roswell Park Cancer Institute, State University of New York at Buffalo

Asher A Chanan-Khan, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Hanxian Huang, MD, PhD Staff Physician, Department of Internal Medicine, Leesburg Regional Medical Center, The Villages Regional Hospital

Hanxian Huang, MD, PhD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine

Disclosure: Nothing to disclose.

Anand B Karnad, MBBS Program Director, Fellowship Programs in Hematology-Oncology, Professor of Medicine, Division of Medical Oncology, Department of Medicine, University of Texas Health Sciences Center, San Antonio

Anand B Karnad, MBBS is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Osler Society, American Society of Hematology, Assocation of Subspecialty Professors, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

Olga Kozyreva, MD Attending Physician, Division of Hematology-Oncology, St Elizabeth's Medical Center; Assistant Professor, Tufts University School of Medicine

Disclosure: Nothing to disclose.

Sarah K May, MD Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC

Disclosure: Nothing to disclose.

from Memorial Sloan-Kettering - Philip Schulman, MD Chief, Medical Oncology, Department of Medicine, Memorial Sloan-Kettering Cancer Center; Clinical Professor, Department of Medicine, New York University School of Medicine

Philip Schulman, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology, and Medical Society of the State of New York

Disclosure: Nothing to disclose.

Karen Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching; Schering Honoraria Speaking and teaching; Cephalon Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Yubao Wang, MD, PhD Fellow, Division of Hematology/Medical Oncology, University of Texas Health Science Center, San Antonio

Yubao Wang, MD, PhD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

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Computed tomography scan in a patient with a large, left-sided axillary mass from which a biopsy was obtained. Biopsy findings were consistent with small noncleaved cell non-Hodgkin lymphoma.
Postchemotherapy computed tomography scan in a patient diagnosed with small noncleaved cell lymphoma (SNCCL) (same patient as in previous image). This image shows regression of a left axillary mass.
Coronal magnetic resonance imaging (MRI) section in a patient with large neck mass (same patient as in previous image). Biopsy findings showed Burkitt-like non-Hodgkin lymphoma (NHL). MRI was performed to assess for cord involvement.
Sagittal magnetic resonance imaging (MRI) section of the neck area showing a large mass invading the cervical spine with epidural encroachment (same patient as in the previous image). MRI was performed to rule out cord compression. The first image shows the gallium scan of this patient that correlates with the site of the tumor.
Right-sided pleural effusion in a patient with small noncleaved cell lymphoma (SNCCL) non-Hodgkin lymphoma.
Hematoxylin and eosin (H&E) stain. Sheets of monotonous-appearing lymphoid cells with one or more prominent nucleoli and an area of pale staining resulting from the presence of benign macrophages reveal a starry sky pattern.
The 2-dimensional flow cytometry demonstrates the highlighted cells to be CD5 negative and CD23 negative as well as lambda negative. Small noncleaved cell lymphoma (SNCCL) cells are typically CD19+, CD20+, CD22+, and CD10+.
 
 
 
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