Anaplastic large cell lymphoma, ALK-negative

ICD-O-3 Morphology

9715/3: Anaplastic large cell lymphoma, ALK-negative / Breast implant-associated anaplastic large cell lymphoma
Effective 2021 and later


for cases diagnosed 2021 and later

Primary Site(s)

No primary site specified

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9702/3.)

ALK- ALCL involved both lymph nodes and extranodal tissue (e.g., the bone, soft tissue, and skin), although extranodal sites are less commonly involved than in ALK+ ALCL.

Most patients present with advanced stage (III-IV) disease, with peripheral and/or abdominal lymphadenopathy and B symptoms.

For breast implant-associated anaplastic large cell lymphomas, the tumor cells may be localized to the seroma cavity or may involve the pericapsular fibrous tissue, sometimes forming a mass. Locoregional lymph nodes may be involved. For this diagnosis, primary site is the breast (C50_)

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.


Not Applicable

Module Rule


Alternate Names

Breast implant-associated anaplastic large cell lymphoma
Seroma-associated anaplastic large cell lymphoma


Anaplastic large cell lymphoma, ALK-negative (ALK-) is defined as a CD30+ T-cell neoplasm that is not reproducibly distinguishable on morphological grounds from ALK-positive (ALK+) ALCL, but lacks ALK protein expression.

ALK-ALCL must be distinguished from primary cutaneous ALCL (C-ALCL), other subtypes of CD30+ T-cell or B-cell lymphoma with anaplastic features, and classic Hodgkin lymphoma (CHL).

Breast implant-associated anaplastic large cell lymphoma is a provisional entity that is a T-cell lymphoma with morphological and immunophenotypic features indistinguishable from those of ALK-negative anaplastic large cell lymphoma (ALCL), arising primarily in association with a breast implant.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing

Genetics Data

TR genes are clonally rearranged


CD2 expression
CD3 expression
CD4 positive
CD30 strongly positive
C43 expressed
Granzyme B expression
Perforin expression
TIA1 expression



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

C84.5 Other and unspecified T-cell lymphomas

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

C84.7 Anaplastic large cell lymphoma, ALK-negative (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)
Peripheral and abdominal lymphadenopathy
Peri-implant effusion (breast)

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Flow cytometry
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation


Epidemiology and Mortality

Age: 40-65 median age, can occur at any age


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: Section: Mature T- and NK-cell neoplasms
Pages: 418-422

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020
Section: ICD-O-3.2 (2020) Morphological Codes