Burkitt cell leukemia

Name
Burkitt cell leukemia
ICD-O-2 Morphology
9826/3
Effective 1992 - 2000
ICD-O-3 Morphology
9826/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C421

Help me code for dx year :

Grade
6 - B-cell
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
The Burkitt leukemia (or acute lymphocytic leukemia FAB L-3) tends to involve the CNS at diagnosis or early during the disease course.

Highly aggressive NHL presenting as acute leukemia; historically classified as small non-cleaved cell lymphoma with solid tumor or nodal mass, as L3 acute lymphoblastic leukemia with > 25% bone marrow involvement. Lymphoma/leukemia recognized as single entity by WHO on basis of shared molecular/genetic features.
Abstractor Notes
The leukemic variant of Burkitt is rare. Only the peripheral blood and bone marrow are involved; there is no lymphadenopathy or bulky disease. CNS tends to be involved at diagnosis or early in the disease process. Burkitt lymphoma is chemosensitive which easily leads to acute tumor lysis syndrome (massive tumor break-down causing sepsis).
Definitive Diagnostic Methods
Bone Marrow biopsy
Immunophenotyping
Peripheral blood smear
Genetics Data
t(8;14) in 80%, t(2;8)(p12;q24) or t(8;22)(q24:q11) in 20%
Immunophenotyping
Bcl-6+
CD5-
CD10+
CD19+
CD20+
CD22+
CD23-
CD79a+
surface IgM+
TdT-
Treatments
Chemotherapy
Stem cell transplant
Transformations to
No Transformations
Transformations from
No Transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.A Mature B-cell leukemia Burkitt-type
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None