Name

B-lymphoblastic leukemia/lymphoma with t(v;11q23.3); KMT2A-rearranged

ICD-O-3 Morphology

9813/3: B Lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 4: Rules PH7, PH8
Usually presents as a leukemia. Organ involvement is possible. Lymphomatous presentation is rare. High incidence of CNS involvement at diagnosis

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

B-ALL/LBL with t(v; 11q23.3)
B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL-rearranged

Definition

B-lymphoblastic leukemia/lymphoma with t(v;11q23.3) is a neoplasm of lymphoblasts committed to the B-cell lineage in which the blasts harbor a translocation between KMT2A (also called MLL) at band 11q23/3 and any of a large number of fusion partners. Leukemias that have deletions of 11q23.3 without KMT2A rearrangement are not included in this group.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes: 9728/3 [lymphoma] or 9836/3 [leukemia].)

The specific etiology of leukemia with MLL translocations is unknown; however, it may occur in utero, with a short latency between the translocation and development of neoplasm. Evidence for this statement includes the fact that these leukemias are frequent in very young infants.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

AFF1 (AF4) on chromosome 4q21
FLT-3 overexpression
KMT2A gene on chromosome 11q23.3
KMT2A MLLT1 fusions
MLLT1 (ENL) on chromosome 19p13
MLLT3 (AF9) on chromosome 9p21.3

Immunophenotyping

CD10-
CD15+
CD19+
CD24-negative pro-B immunophenotype
NG2 homologue encoded by CSPG4

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

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)

Diagnostic Exams

Progression and Transformation

None

Epidemiology and Mortality

Age: may occur in utero, frequently found in young infants
Incidence: most common leukemia in infants <1 year of age
Survival: poor prognosis (especially for infants less than 6 months of age)

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: 203-204

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