Name

T-cell/histiocyte-rich large B-cell lymphoma

ICD-O-3 Morphology

9688/3: T-cell/histiocyte-rich large B-cell lymphoma
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

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

Grade

Not Applicable

Module Rule

None

Alternate Names

B-cell lymphoma rich in T-cells and simulating Hodgkin disease
Histiocyte-rich large B-cell lymphoma
Histiocyte-rich/T-cell rich large B-cell lymphoma
T-cell rich/histiocyte-rich large B-cell lymphoma
THRLBCL

Definition

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is characterized by a limited number of scattered, large B cells embedded in a background of abundant T cells and histiocytes. THRLBCL may arise de novo.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later.)
THRLBCL mainly affects the lymph nodes but bone marrow, liver and spleen involvement are frequently found at diagnosis.

Most patients present at advanced stages (Stage IV with bone marrow involvement). This disease is often refractory to chemotherapy. Most frequently treated with CHOP.

If cells are EBV positive, need to reconsider diagnosis of THRLBCL (should instead be considered a type of DLBCL). This is an aggressive lymphoma.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Clonal Ig rearrangements

Immunophenotyping

BCL2 expression
BCL6 positive
CD3 positive
CD5 positive
CD19 expression
CD20 expression
CD79a expression
CD68 positive
CD163 positive
EMA expression

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations from

None

Corresponding ICD-9 Codes

200.7 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)
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

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: primary middle age (12-61 year range)
Incidence: <10% of all diffuse large B-cell lymphoma (DLBCL)
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: 298-299

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