Diffuse large B-cell lymphoma (DLBCL)

Name
Diffuse large B-cell lymphoma (DLBCL)
ICD-O-2 Morphology
9680/3
Effective 1992 - 2000
ICD-O-3 Morphology
9680/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
Module 6: PH11, PH13
Alternate Names
Age-related EBV+ lymphoproliferative disorder
Anaplastic large B-cell lymphoma
Angioendotheliomatosis [OBS] see 9712/3
Angiotropic lymphoma [OBS] see 9712/3
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
Diffuse large B-cell lymphoma, NOS
Diffuse large B-cell lymphoma, NOS anaplastic variant
Diffuse large B-cell lymphoma, NOS centroblastic variant
Diffuse large B-cell lymphoma, NOS immunoblastic variant
Diffuse large B-cell lymphoma/Intravascular large B-cell lymphoma
DLBCL
DLBCL associated with chronic inflammation
EBV Positive DLBCL of the elderly
EBV-associated B-cell lymphoproliferative disorder of the elderly
Germinal centre B-cell-like GCB
Histiocyte-rich large B-cell lymphoma [OBS] see 9688/3
Hodgkin-like anaplastic large cell lymphoma
Intravascular B-cell lymphoma [OBS] see 9712/3
Intravascular large B-cell lymphoma [OBS] see 9712/3
Lymphomatoid granulomatosis grade 3
Malignant lymphoma, centroblastic, diffuse, NOS
Malignant lymphoma, centroblastic, NOS
Malignant lymphoma, histiocytic, diffuse, NOS
Malignant lymphoma, histiocytic, NOS [OBS]
Malignant lymphoma, large B-cell, diffuse, centroblastic, NOS
Malignant lymphoma, large B-cell, diffuse, NOS
Malignant lymphoma, large cell, cleaved [OBS]
Malignant lymphoma, large cell, cleaved and non cleaved [OBS]
Malignant lymphoma, large cell, cleaved, diffuse
Malignant lymphoma, large cell, diffuse, NOS [OBS]
Malignant lymphoma, large cell, large B-cell, NOS
Malignant lymphoma, large cell, non cleaved, NOS
Malignant lymphoma, large cell, noncleaved, diffuse, NOS [OBS]
Malignant lymphoma, large cell, NOS
Malignant lymphoma, large cleaved cell, NOS [OBS]
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
Primary DLBCL of the CNS
Primary intraoccular lymphoma
Richter syndrome
Splenic EBV-associated B-cell lymphoproliferative disorder
T-cell rich B-cell lymphoma [OBS] see 9688/3
T-cell rich/histiocytic rich large B-cell lymphoma [OBS] see 9688/3
Definition
Lymphoma with diffuse proliferation of large neoplastic B lymphoid cells with nuclear size exceeding macrophage nuclei, more than twice size of normal lymphocytes. Normal architecture of node or extranodal tissue replaced in diffuse pattern. Morphologic variants: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.
Abstractor Notes
Diffuse large B-cell lymphoma has several variants, all of which are coded to 9680/3. Those variants include: primary DLBCL of the CNS; primary cutaneous DLBCL, leg type; EBV positive DLBCL of the elderly; DLBCL associated with chronic inflammation; B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma; and B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma.

Code the primary site to skin when there is skin infiltration with large B-cell lymphoma, B-cell lymphoma or large cell lymphoma and the lymphoma is limited to skin and the regional lymph nodes. If there is involvement of lymph node(s) that are not regional for the skin site involved, or involvement of bone marrow or organ(s), do not code skin as the primary site. In this situation, skin is probably a metastatic site.

DLBCL of the CNS: Approximately 80% are supratentorial. 20-50% have multiple lesions. Approximately 20% have intraocular lesions. Many of the intraocular lesions are contralateral, and the patient may develop parenchymal lesions. Sporadic systemic recurrences may affect any organ but relatively frequently the testis and breast.

B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt lymphoma: patients present with lymphadenopathy or mass lesions in extranodal sites. Some patients have a leukemic presentation.

DLBCL associated with chronic inflammation: common sites are pleural cavity, bone (especially femur) joining, and periarticular soft tissue. Tumor mass >10 cm in most cases.

Primary cutaneous DLBCL, leg type: these lymphomas usually affect the lower leg but 10-15% arise in other sites. Patient presents with red or bluish-red tumors on one or both of the lower legs. Frequently disseminates to other sites.

EBV pos DLBCL of elderly: 70% have extranodal involvement, most commonly skin, lung, tonsil, and stomach with or without LN involvement. The remaining 30% present with LN involvement only.

B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt: more than half present with widespread extranodal disease. May have lesions in ileocecal region or jaws. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal site.

B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma: most commonly present with large anterior mediastinal mass with or without involvement of supraclavicular LN. Less commonly, other peripheral LN groups are involved. May be direct extension to lung as well as spread to liver and/or bone marrow.

Richter syndrome is a term that is used to describe the transformation from either CLL/SLL or hairy cell leukemia to DLBCL.
Definitive Diagnostic Methods
Cytology (for primary CNS lymphoma only)
Genetics testing
Histological confirmation
Immunophenotyping
Genetics Data
Bq24/MYC translocations
Immunoglobulin genes clonally rearranged and hypermutated
Mutation BCL6
Mutation MYC
Mutation PAX5
Mutation PIM1
Mutation RhoH/TTFn
Mutation TP53
Translocations iinvolving c-MYC, BCL6, and IgH genes
Immunophenotyping
BCL2+
BCL6+
BCL6-
CD10+
CD15+
CD20+
CD22+
CD30+
CD79a+
EBNA-2+
Fox-P1+
IRF4/MUM1+
LMP-1+
Surface4 or cytoplasmic immunoglobulin (Ig) is absent
Treatments
Chemotherapy
Hormone
Radiation
Stem cell transplant
Transformations to
No Transformations
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None