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Effective
1978 - 1991
Effective
1992 - 2000
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
See Abstractor Notes and Module 7
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Patients may present with nodal or extranodal disease. The most common extranodal site is the gastrointestinal site (stomach and ileocecal region). Other common sites of extranodal presentation include the bone, testes, spleen, Waldeyer ring, salivary glands, thyroid, liver, kidneys, and adrenal glands.
Patients usually present with a rapidly enlarging tumor mass at single or multiple nodal or extranodal sites. Many patients are asymptomatic, but B symptoms may be present. Specific localizing symptoms may be present and are highly dependent on the site of extranodal involvement.
For more information on lymphoma, see the NCI website: http://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq#section/_1 or http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_129
Patients usually present with a rapidly enlarging tumor mass at single or multiple nodal or extranodal sites. Many patients are asymptomatic, but B symptoms may be present. Specific localizing symptoms may be present and are highly dependent on the site of extranodal involvement.
For more information on lymphoma, see the NCI website: http://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq#section/_1 or http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_129
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
Module 6: PH11, PH13
Alternate Names
Activated B-cell subtype
Age-related EBV+ lymphoproliferative disorder
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
DLBCL
DLBCL associated with chronic inflammation
Double hit lymphoma
EBV Positive DLBCL of the elderly
EBV-associated B-cell lymphoproliferative disorder of the elderly
Germinal centre B-cell-like GCB
Germinal centre B-cell subtype
Malignant lymphoma, centroblastic, NOS
Malignant lymphoma, noncleaved, NOS
Non-germinal centre B-cell-like; non-GCB
PCLBCL leg type
PCNSL
PIOL
Primary CNS lymphoma
Primary cutaneous DLBCL, leg type (C447)
Primary DLBCL of the CNS (C70_, C71_, C72_)
Primary intraocular lymphoma
Pyothorax-associated lymphoma
Richter syndrome
Splenic EBV-associated B-cell lymphoproliferative disorder
Triple hit lymphoma
Vitreoretinal lymphoma (C69_)
Definition
Diffuse large B-cell lymphoma (DLBCL) is a neoplasm of medium or large B lymphoid cells whose nuclei are the same size as, or larger than, those of normal macrophages, or more than twice the size of those of normal lymphocytes, with a diffuse growth pattern.
Morphological, biological, and clinical studies have divided DLBCLs into morphological variants, molecular subtypes, and distinct disease entities.
Diffuse large B-cell lymphoma associated with chronic inflammation is a lymphoid neoplasm occurring in the setting of longstanding chronic inflammation and showing association with EBV. Most cases involve body cavities or narrow spaces. Pyothorax-associated lymphoma (PAL) is the protypical form, developing in the pleural cavity of patients with longstanding pyothorax.
EBV-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS), is an EBV-positive clonal B-cell lymphoid proliferation. Excluded from this category are cases of lymphomatoid granulomatosis, cases with evidence of acute or recent EBV infection, other well-defined lymphomas that may be EBV-positive (such as plasmablastic lymphoma and DLBCL associated with chronic inflammation), and EBV-positive mucocutaneous ulcer. This disease was formerly designated as EBV-positive DLBCL of the elderly. The elderly designation has been removed.
Fibrin-associated diffuse large B-cell lymphoma is an unusual form of diffuse large B-cell lymphoma associated with chronic inflammation. It is not mass forming and does not directly produce symptoms, but is discovered incidentally on histological examination of surgical pathology specific excised for various pathologies other than lymphomas.
High-grade B-cell lymphoma (HGBL) is a group of aggressive, mature B-cell lymphomas . There are two categories of HGBL.
1) HGBL with MYC and BCL2 and/or BCL6 rearrangements, encompasses all B-cell lymphomas that have a MYC (8q24) rearrangement in combination with a BCL2 (18q21) and or a BCL6 (3q27) rearrangement, i.e. the so-called double-hit and triple-hit lymphomas.
2) HGBL, NOS encompasses cases that either have features intermediate between DLBCL and BL, or appear blastoid, but by definition do not harbor a genetic double hit.
Primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, is a PCLBCL composed exclusively of centroblasts and immunoblasts, most commonly arising within the leg.
Primary diffuse large B-cell lymphoma (DLBCL) of the CNS is defined as DLBCL arising within the brain, spinal cord, leptomeninges or eye. Excluded are lymphomas of the dura, intravascular large B-cell lymphomas, lymphomas with evidence of systemic disease or secondary lymphomas, and all immunodeficiency-associated lymphomas.
Morphological, biological, and clinical studies have divided DLBCLs into morphological variants, molecular subtypes, and distinct disease entities.
Diffuse large B-cell lymphoma associated with chronic inflammation is a lymphoid neoplasm occurring in the setting of longstanding chronic inflammation and showing association with EBV. Most cases involve body cavities or narrow spaces. Pyothorax-associated lymphoma (PAL) is the protypical form, developing in the pleural cavity of patients with longstanding pyothorax.
EBV-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS), is an EBV-positive clonal B-cell lymphoid proliferation. Excluded from this category are cases of lymphomatoid granulomatosis, cases with evidence of acute or recent EBV infection, other well-defined lymphomas that may be EBV-positive (such as plasmablastic lymphoma and DLBCL associated with chronic inflammation), and EBV-positive mucocutaneous ulcer. This disease was formerly designated as EBV-positive DLBCL of the elderly. The elderly designation has been removed.
Fibrin-associated diffuse large B-cell lymphoma is an unusual form of diffuse large B-cell lymphoma associated with chronic inflammation. It is not mass forming and does not directly produce symptoms, but is discovered incidentally on histological examination of surgical pathology specific excised for various pathologies other than lymphomas.
High-grade B-cell lymphoma (HGBL) is a group of aggressive, mature B-cell lymphomas . There are two categories of HGBL.
1) HGBL with MYC and BCL2 and/or BCL6 rearrangements, encompasses all B-cell lymphomas that have a MYC (8q24) rearrangement in combination with a BCL2 (18q21) and or a BCL6 (3q27) rearrangement, i.e. the so-called double-hit and triple-hit lymphomas.
2) HGBL, NOS encompasses cases that either have features intermediate between DLBCL and BL, or appear blastoid, but by definition do not harbor a genetic double hit.
Primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, is a PCLBCL composed exclusively of centroblasts and immunoblasts, most commonly arising within the leg.
Primary diffuse large B-cell lymphoma (DLBCL) of the CNS is defined as DLBCL arising within the brain, spinal cord, leptomeninges or eye. Excluded are lymphomas of the dura, intravascular large B-cell lymphomas, lymphomas with evidence of systemic disease or secondary lymphomas, and all immunodeficiency-associated lymphomas.
Definitive Diagnostic Methods
Cytology (for primary CNS lymphoma only)
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
BCL2 (18q21.3 gain/amplification)
B2M mutation
CARD11 mutation
CD58 mutation
CD274/PDCD1LG2 (9p24.1 gains/amplification )
CD79B mutation
CREBBP mutation
EZH2 mutation
GNA13 mutation
KMT2D (also called MLL2) mutation
MEF2B mutation
MYD88 mutation
PRDM1 (6q21 deletion)
PTPN1 mutation
REL (2p16 gain/amplification)
SGK1 mutation
SOCS1 mutation
STAT6 mutation
TNFRSF14 (1p36;32 deletion)
TP53 mutation
3q27 gain/amplication
Immunophenotyping
BCL2+ (expression/positive)
BCL6+ (expression/positive)
CD5+ (expression/positive)
CD10+ (expression/positive)
CD15+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
CD30+ (expression/positive)
CD79a+ (expression/positive)
FOXP1+ (expression/positive)
IRF4/MUM1+ (expression/positive)
LMO2+ (expression/positive)
PAX5+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy
Transformations to
None
Transformations from
Same Primaries
Corresponding ICD-9 Codes
200.7 Large cell lymphoma
200.5 Primary central nervous system lymphoma
Corresponding ICD-10 Codes
C83.3 Non-Hodgkin lymphoma large cell (diffuse)
C83.9 Diffuse non-Hodgkin lymphoma, unspecified
C83.6 Non-Hodgkin lymphoma undifferentiated (diffuse)
Corresponding ICD-10-CM Codes (U.S. only)
C83.3 Diffuse large B-cell lymphoma (effective October 01, 2015)
C83.9 Non-follicular (diffuse) lymphoma, unspecified (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
Blood chemistry studies
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: 70 years median age (occur in children and adults)
Incidence: 25-30% of adult NHL's (western countries)
Sex: slight male predominance
Survival: 5 year survival ~62% without bone marrow involvement; 5 year survival 10% with bone marrow involvement
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: 291--297, 300-306, 309-311, 335-341
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 291--297, 300-306, 309-311, 335-341
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
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
