Search Database ICD-O-3 Code Lists

Name

B-lymphoblastic leukemia/lymphoma, NOS

ICD-O-3 Morphology

9811/3: B lymphoblastic leukemia/lymphoma, NOS
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)

Coding Manual: Hematopoietic Coding Manual (PDF)

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.

In most cases, the NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis. If no information is found in the medical record, follow-back to the attending physician should be done.

More specific B-lymphoblastic leukemia/lymphomas are:
1. 9812/3: B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2); BCR-ABL1
2. 9813/3: B-lymphoblastic leukemia/lymphoma with t(v;11q23.3); KMT2A-rearranged
3. 9814/3: B-lymphoblastic leukemia/lymphoma with t(12;21)(p13.2;q22.1); ETV6-RUNX1
4. 9815/3: B lymphoblastic leukemia/lymphoma with hyperdiploidy
5. 9816/3: B lymphoblastic leukemia/lymphoma with hypodiploidy (hypodiploid ALL)
6. 9817/3: B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.1); IGH/IL3
7. 9818/3: B-lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); TCF3-PBX1
8. 9819/3: B-lymphoblastic leukemia/lymphoma, BCR-ABL1 like (2021+)

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

Acute lymphoblastic leukemia
ALL
BLL
B-ALL
B-cell lymphoma/leukemia (immature) (see 9591/3 for mature))
B-LBL
B-lymphoblastic leukemia/lymphoma, iAMP21
B-lymphoblastic leukemia/lymphoma, not otherwise specified (NOS)
c-ALL
Common ALL
Common lymphoblastic leukemia
Common precursor B ALL
Common precursor B-lymphoblastic leukemia
B-cell lymphoma/leukemia (immature), NOS (see 9591/3 for mature)
Pre-B lymphoblastic leukemia
Precursor cell lymphoblastic leukemia
Precursor B-cell lymphoblastic leukemia, NOS
Precursor B-cell lymphoblastic lymphoma
Pre-B ALL
Pre-pre-B ALL
Pre-pre-B lymphoblastic leukemia
Pro-B lymphoblastic leukemia
Pro-B ALL

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).

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

Clonal rearrangements of IGH
T-cell receptor rearrangements

Immunophenotyping

CD10+ (expression/positive)
CD19+ (expression/positive)
CD22+ (expression/positive)
CD24+ (expression/positive)
CD79a+ (expression/positive)
PAX5+ (expression/positive)
TdT+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-9 Codes

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

Corresponding ICD-10 Codes

C83.5 Non-Hodgkin 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, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

National Cancer Institute
Section: General Information About Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq
Glossary