B lymphoblastic leukemia/lymphoma, NOS

Name
B lymphoblastic leukemia/lymphoma, NOS
ICD-O-3 Morphology
9811/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Most common sites of involvement: B-ALL (bone marrow, peripheral blood, central nervous system, lymph nodes, spleen, liver and testis in males), B-LBL (skin, soft tissue, bone and lymph nodes)

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Grade
6 - B-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
ALL
B-ALL
B-LBL
B lymphoblastic leukemia/lymphoma, NOS
Definition
A neoplasm of precursor cells (lymphoblasts) committed to the B-cell lineage, typically composed of small to medium sized blast cells.

Patients with B-LBL without leukemia are usually asymptomatic and most have limited stage disease.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see codes: 9728/3 [lymphomas] or 9836/3 [leukemia].)

B-Acute lymphoblastic leukemia (B-ALL)
Bone marrow is involved in all cases and peripheral blood is usually involved. Extramedullary involvement is frequent,

B-Lymphoblastic lymphoma (B-LBL)
The most frequent sites of involvement are the skin, soft tissue, bone and lymph nodes.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
Clonal DJ rearrangements of the IGH@ gene
T-cell receptor rearrangements
Immunophenotyping
CD10+
CD19+
CD22+
CD24+
CD79a+
PAX5+
TdT+
Treatments
Chemotherapy
Radiation
Stem cell transplant
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 (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Immunhistochemistry
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biposy
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: primarily disease of children (75% of cases under 6 years age)
Incidence: 1-4.74/100,000 per year worldwide, 10% of lymphoblastic lymphomas
Sex. slight male predominance
Survival: ~80% of children cured; less than 50% of adults cured