Name

B-lymphoblastic leukemia/lymphoma, NOS

ICD-O-3 Morphology

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)

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

ALL
BLL
B-ALL
B-LBL
B-lymphoblastic leukemia/lymphoma, iAMP21
B-lymphoblastic leukemia/lymphoma, not otherwise specified (NOS)
Common precursor B-lymphoblastic leukemia

Definition

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is a neoplasm of precursor lymphoid cells committed to the B-cell lineage, typically composed of small to medium-sized blast cells with scant cytoplasm, moderately condensed to dispersed chromatin, and inconspicuous nucleoli, involving bone marrow and blood (B-ALL) and occasionally presenting with primary involvement of nodal or extranodal site (B-LBL).

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. Extracellular 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 rearrangements of IGH
T-cell receptor rearrangements

Immunophenotyping

CD10+
CD19+
CD22+
CD24+
CD79a+
PAX5+
TdT+

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.1 Lymphosarcoma (Lymphoma presentation)
204.0 Acute lymphoid leukemia (Leukemia presentation)

Corresponding ICD-10 Codes

C83.5 Diffuse non-Hodgkin's lymphoma, lymphoblastic (diffuse) (Lymphoma presentation)
C91.0 Acute lymphoblastic leukemia (Leukemia presentation)

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

C83.5 Lymphoblastic (diffuse) lymphoma (Lymphoma presentation) (effective October 01, 2015)
C91.0 Acute lymphoblastic leukemia [ALL] (Leukemia presentation) (effective October 01, 2015)

Signs and Symptoms

Anemia
Arthralgias
Bone pain
Elevated white blood cell (WBC) count
Hepatomegaly
Lymphadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunhistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biposy
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

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: Precursor lymphoid neoplasms
Pages: 200-202, 208

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 Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq
Glossary