Name

ALK-positive large B-cell lymphoma

ICD-O-3 Morphology

9737/3: ALK-positive large B-cell lymphoma
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, mediastinal mass, nasopharynx, tongue, stomach, bone and soft tissues.

Grade

Not Applicable

Module Rule

None

Alternate Names

ALK-positive LBCL
ALK-positive plasmablastic B-cell lymphoma [OBS]
Large B-cell lymphoma expressing the ALK kinase and lacking the t(2;5) translocation [OBS]
LBCL

Definition

ALK-positive large B-cell lymphoma (LBCL) is an aggressive neoplasm of ALK-positive monomorphic large immunoblast-like B cells, which usually have a plasma cell phenotype.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9684/3.)

Most patients present with Stage III/IV. Because these tumors are negative for CD20 antigen, they are insensitive to rituximab.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping
Karyotyping

Genetics Data

CLTC-ALK fusion gene
Immunoglobulin genes are clonally rearranged
Phospho-STAT3
SQSTM1 or SEC31A fusion
t(2;17)(p23;q23)

Immunophenotyping

ALK protein positive
CD3-
CD20-
CD30-
CD45 weak or negative
CD79a-
CD138+
CLTC-ALK fusion protein expression
Cytoplasmic staining with other ALK translocations
EMA
IRF4/MUMI positive
NPM1-ALK fusion protein with nucleolar ALK staining
PAX5-
PRDm1 (also known as BLIMP1)
VS38+

Treatments

Chemotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.6 Anaplastic large cell lymphoma

Corresponding ICD-10 Codes

C83.3 Diffuse non-Hodgkins lymphoma, large cell (diffuse)

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

C83.3 Diffuse large b-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Mediastinal mass
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Progression and Transformation

None

Epidemiology and Mortality

Age: 36 years median age (9-70 years range)
Incidence: <1% of DLBCL lymphomas
Sex: male predominance
Survival: median survival 11 months; >156 months survival for children

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: Mature B-cell neoplasms
Pages: 319-320

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 Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary