Name

Burkitt cell leukemia

ICD-O-2 Morphology

9826/3: Burkitt cell leukemia
Effective 1992 - 2000

ICD-O-3 Morphology

9826/3: Burkitt cell leukemia
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

C421

Grade

Not Applicable

Module Rule

None

Alternate Names

Acute leukemia, Burkitt type [OBS]
Acute lymphoblastic leukemia, mature
Acute lymphoblastic leukemia, mature B-cell type
Acute lymphocytic leukemia FAB L-3
B-ALL [OBS] see 9811/3
Burkitt leukemia
FAB L3 [OBS]

Definition

Burkitt lymphoma (BL) (9687/3) is a highly aggressive but curable lymphoma that often presents in extranodal sites or as an acute leukemia. It is composed of monomorphic medium-sized B cells with basophilic cytoplasm and numerous mitotic figures. The frequency of EBV infection varies according to the epidemiological subtype of BL.

The leukemic phase can be observed in patients with bulky disease, but only rare cases, present purely as leukemia with peripheral blood and bone marrow involvement. Burkitt leukemia tends to involve the CNS at diagnosis or early in the disease.

Abstractor Notes

Bone marrow involvement, without peripheral blood involvement, does not qualify for the leukemic phase for Burkitt's. There must be bone marrow involvement and peripheral blood involvement to qualify as the leukemic phase.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

Clonal IG rearrangements
MYC translocation at band 8q24 to the IGH region on chromosome 14q32
IGK locus on 2p12 [t(2;8]
IGL locus on 22q11 [t(8;22]

Immunophenotyping

BCL2-
BCL6+
CD3-
CD5-
CD10+
CD19+
CD20+
CD22+
CD23-
CD38+
CD43+
CD56+
CD77+
CD79a+
CD138-
Ki67+
MYC protein strong expression
TCL1 strongly expressed in pediatric BL
TdT-

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

204.8 Other lymphoid leukemia

Corresponding ICD-10 Codes

C91.7 Other lymphoid leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C91.A Mature B-cell leukemia Burkitt-type (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph node
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 330--334

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary