Name

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma

ICD-O-3 Morphology

9596/3: Composite Hodgkin and non-Hodgkin, NOS
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C770-C779
Lymph nodes (C770-C779) are the usual primary sites; however, involvement in other sites is possible. If you have confirmation that the only involved site is something other than the lymph nodes, than code to that primary site. See Module 7.

Grade

Not Applicable

Module Rule

M5 Module 6: PH 14

Alternate Names

Compositive Hodgkin and non-Hodgkin lymphoma
Composite lymphoma
Grey zone lymphoma
GZL
Hodgkin-like anaplastic large cell lymphoma [OBS]
Large B-cell lymphoma with Hodgkin features
Mediastinal grey-zone lymphoma
MGZL

Definition

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classic Hodgkin lymphoma (CHL) is a B-cell-lineage lymphoma that demonstrates overlapping clinical, morphological, and/or immunophenotypic features between CHL and DLBCL. These lymphomas are most commonly associated with mediastinal disease, but similar cases have been reported in peripheral lymph node groups as the primary site. Mediastinal cases are often referred to as mediastinal grey-zone lymphoma (MGZL) and non-mediastinal cases are grey-zone lymphoma (GZL).

Abstractor Notes

Prior to 2010, the term for this code was composite Hodgkin and non-Hodgkin lymphoma. In the 2008 WHO, the term used to describe this same disease is B-cell lymphoma, unclassifiable, with features indeterminate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma. The change in terminology reflects current clinical knowledge that the non-Hodgkin portion of this lymphoma has proven to be a B-cell immunophenotype.

The most common presentation is with a large anterior mediastinal mass, with or without involvement of supraclavicular lymph nodes. There may be spread to the lung by direct extension, as well as spread to liver, spleen, and bone marrow. Non-lymphoid organs are rarely involved.

Definitive Diagnostic Methods

Histologic confirmation
Immunophenotyping

Genetics Data

PDCD1LG2 (also called PDL2) loci at 9p24.1
MYC gains
9p24.1 gains/amplifications

Immunophenotyping

ALK-
BCL6 variably positive
BOB1 expressed
CD3+
CD4+
CD10-
CD15 expressed
CD20 weak or variably positive
CD30+
CD45+
CD79a+
OCT2 expressed
PAX5 expressed

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.8 Other named variants, lymphoma
202.8 Other lymphomas

Corresponding ICD-10 Codes

C83.9 Diffuse non-Hodgkin's lymphoma, unspecified
C85.9 Non-Hodgkin's lymphoma, unspecified type

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

C83.9 Non-follicular (diffuse) lymphoma, unspecified (effective October 01, 2015)
C85.1 Unspecified B-cell lymphoma (effective October 01, 2015)
C85.9 Non-Hodgkin's lymphoma, unspecified (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Large 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

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

Progression and Transformation

None

Epidemiology and Mortality

Age: 20-40 years most common (13-70 years range)
Sex: male predominance

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: 342-344

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