Name

Anaplastic large cell lymphoma, ALK-positive

ICD-O-2 Morphology

Effective 1992 - 2000

ICD-O-3 Morphology

9714/3: Anaplastic large cell lymphoma, T cell and Null cell type
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, skin, bone, soft tissue, lung, and liver. Less common sites of involvement: GI, CNS.

Grade

Not Applicable

Module Rule

None

Alternate Names

ALCL, ALK+
Anaplastic large cell lymphoma, CD30+
Anaplastic large cell lymphoma, small cell variant
Ki-1 lymphoma [OBS]
Large cell (Ki1+) lymphoma [OBS]

Definition

Anaplastic large cell lymphoma (ALCL), ALK-positive (ALK+) is a T-cell lymphoma consisting of lymphoid cells that are usually large and have abundant cytoplasm and pleomorphic, often horseshoe-shaped nuclei, with a chromosomal translocation involving the ALK gene and expression of ALK protein and CD30.

Abstractor Notes

Anaplastic large cell lymphoma, ALK-positive (ALCL, ALK+) frequently involves both lymph nodes and extranodal sites.The most commonly involved extranodal sites include skin, bone, soft tissue, lung, and liver. Involvement of the GI system and CNS is rare. Mediastinal involvement is less frequent than in Classical Hodgkin lymphoma.

Bone marrow involvement occurs in approximately 10-30% depending on the tests performed. The small cell variant may have a leukemic presentation with peripheral blood involvement.

Patients often have B-symptoms, especially high fever.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

t (2;5) (p23;q35)
TR genes are clonally rearranged

Immunophenotyping

CD2 positive
CD4 positive
CD5 positive
CD25 positive
CD30 positive
CD43 expression
CD45 positive
CD45RO positive
EMA positive

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy

Transformations to

None

Corresponding ICD-9 Codes

200.6 Anaplastic large cell lymphoma

Corresponding ICD-10 Codes

C85.7 Other specified types of non-Hodgkin's lymphoma

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

C84.6 Anaplastic large cell lymphoma, ALK-positive (effective October 01, 2015)

Signs and Symptoms

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

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: most frequently occurs in 30's (occurs in children and adults)
Incidence: 3% of adult and 10-20% of childhood non-Hodgkin lymphomas
Sex: slight male predominance
Survival: 5 year survival 60-80%

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 T- and NK-cell neoplasms
Pages: 413-418

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