An official website of the United States government
Home
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
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
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.
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Histiocyte-rich large B-cell lymphoma
Histiocyte-rich/T-cell rich large B-cell lymphoma
T-cell rich B-cell lymphoma
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.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal Ig rearrangements
Immunophenotyping
BCL2+ (expression/positive)
BCL6+ (expression/positive)
CD3+ (expression/positive)
CD5+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD79a+ (expression/positive)
CD68+ (expression/positive)
CD163+ (expression/positive)
EMA+ (expression/positive)
Treatments
Chemotherapy
Hormone therapy
Radiation therapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
200.7 Large cell lymphoma
Corresponding ICD-10 Codes
C83.3 Non-Hodgkin 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
Fatigue
Fever (for no known reason)
Hepatomegaly
Malaise
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Splenomegaly
Weight loss (for no known reason)
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: primary middle age (12-61 year range)
Sex: male predominance
Survival: aggressive lymphoma, poor survival
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
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, Second Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
