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

Name
T-cell/histiocyte-rich large B-cell lymphoma
ICD-O-3 Morphology
9688/3
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

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Grade
6 - B-cell
Module Rule
None
Alternate Names
Histiocyte-rich large B-cell lymphoma
Histiocyte rich/T-cell rich large B-cell lymphoma
Large B-cell lymphoma rich in T-cells and simulating Hodgkin disease
T-cell rich/histiocyte-rich large B-cell lymphoma
THRLBCL
Definition
This neoplasm is characterized by a limited number of scattered, large, atypical B cells embedded in a background of abundant T cells and frequently histiocytes which are useful for diagnosis.
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 rearrangement of IG genes in tumor B-cells
Immunophenotyping
BCL2
BCL6
CD3+ T-cells
CD5+ T-cells
CD68+ histiocytes
EMA
Treatments
Chemotherapy
Hormone
Radiation
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 (effective October 1, 2015 U.S. only)
C83.3 Diffuse large B-cell lymphoma
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