3. FEATURES
• Follicular lymphoma is the most
common form of indolent NHL in the
United States
• Middle age men and women equally.
• Arise from germinal center B cells.
• Strongly associated with
translocation involving BCL2
4. HALLMARK
• Translocation( 14; 18)
– This translocation is seen in most
but not all follicular lymphomas
– Leads to overexpression of BCL2
protein.
– BCL2, = is an antagonist of
apoptotic cell death and appears
to promote the survival of
follicular lymphoma cells.
5. • 10% show Peripheral blood
involvement sufficient to produce
lymphocytosis
(usually <20,000/mm3 )
• 85% have Bone marrow involvement
– Paratrabecular lymphoid aggregates.
• Splenic white pulp and hepatic
portal triads are also frequently
involved.
Features
8. • Express CD19, CD20, CD10
– Like Normal follicular center B cells,
• CD5 is NOT Expressed
– In contrast to CLL and SLL and mantle cell
lymphoma, CD5 is expressed.
• OverExpression of BCL2 protein - > 90%
– Versus Normal Follicular center B cells,
which are BCL2 negative
Immunophenotype and Genetics
9. Clinical Features.
• Painless lymphadenopathy, which is
frequently generalized.
• Uncommon Involvement of
extranodal sites
– GIT, CNS, Testis
• Often follows an indolent waxing
and waning course.
10. Survival
• Overall median survival is 7 to 9
years
– Is not improved by aggressive
therapy
– The usual clinical approach is to
palliate patients with low-dose
chemotherapy or radiation when
they become symptomatic.
11. Transformation
• Retain t(14;18)
• Somatic Hypermutation promote
transformation
• Occurs in 30 to 50% of follicular
lymphomas,
– Most commonly to diffuse large B-cell
lymphoma.
• Median survival is less than 1 year
after transformation.
13. • Most common form of NHL
• 60-70%
• Aggressive lymphoid neoplasm
• M>F , Median age 60y/o
DIFFUSE LARGE B-CELL
LYMPHOMA
14. • Rapidly enlarging mass
• Often Symptomatic
• Arise in any site
– Waldeyer ring, Oropharyngeal LN,
Tonsils
– Liver, spleen
• Localized Disease with extranodal
involvement
• Rarely present as leukemia
Features
15. Immunophenotype
• Mature B cell
• Express CD19 & CD20
• Variably Express Germinal Center
Markers
• Have surface Ig
• Negative Tdt
16. Molecular Pathogenesis
• 30% Dysregulation of BCL6
– Repress germinal center B-cell
Differentiation Growth Arrest
Holds cell in Undifferentiated
Proliferative state
– Silence the expression of p53
• Prevent the activation of DNA repair
mechanism
20. Therapy
• 60-80% Complete remission with
combination Chemotherapy
– 50% remain free from disease for years
• Immunotherapy with Anti-CD20 improves outcome
especially elderly
21. Subtype
• Immunedeficiency-associated large B
cell Lymphoma
– T cell immunodeficiency ( HIV )
– (+) EBV Neoplastic B cell
– Restoration of immunity
• Regression of proliferation
23. BURKITTS LYMPHOMA
3 TYPES
1. African ( Endemic )
2. Sporadic ( Non-endemic )
3. Aggressive lymphoma
occuring in HIV patients
o Histologically identical
o Genotype & virologic difference
o CD10 Usually seen
24. Features
o Cell of origin
o Germinal center Bcell
o African LATENTLY INFECTED
w/ EBV
o All forms associated
o Translocations c-myc gene on
Chromosome 8 with IgH [t(8,14)]
o Commonly
25. Clinical features
• Adolescent or Young Adult w/ jaw or
extranodal abdominal mass
• Very aggressive
• Respond well to chemotx
• Outcome guarded in Older adults
• UNCOMMON BM or peripheral blood
26. Clinical features
Endemic
• Often Mandibular
mass
• Unusual predilection
to abdominal viscera
– Kidneys
– Ovary
– Adrenals
Sporadic
• Often as
Abdominal Mass
– Ileocecal
– Peritoneum
33. Features
• LOW grade lesions
• Encompass a heterogenous group of B cell
tumors
• Arise in LN, Spleen, Extranodal Tissues
• Tumor cell resemble normal Marginal Zone
B cells
• Initially recognized at mucosal sites
– MALTOMA
41. • Multiple bone involvement
• Can also spread to LN & Extranodal
• 1% in Western countries
• Higher incidence
• Men>Women
• Older Patients
• Radiation exposure
• African decent
MULTIPLE MYELOMA
42. • Pathogenesis
– IL-6
• Proliferation of tumor cells are DEPENDENT on
Cytokione
• Active Disease and Poor Prognosis
– MIP 1 alpha & RANK Ligand
• Mediate Bone Destruction
• Karyotyping
– Deletions of 13q
– IgH
MULTIPLE MYELOMA
45. • Laboratory
– High M proteins Rouleaux
• 55% IgG Monoclonal Ab
– Proliferation of Neoplastic plasma cells
• 30% of bone marrow cellularity
(Plasma cell Leukemia )
– Bence Jones proteins in urine
• Myeloma kidney
• Seen in 60-80%
• Clinicopathologic Dx
– Correlation of X-ray & Laboratory Findings
MULTIPLE MYELOMA
49. Clinical Features
• Hematologic findings
– Normocytic anemia with rouleaux
– Prolonged bleeding due to defect in
platelet aggregation
• Radiculopathy due to bone
compression and vertebral fracture
• Recurrent infection – Most common
cause of death
52. Solitary Myeloma
• Lesions either in the Bone or Soft
Tissue
– Axial Skeleton
– Lungs, Oropharynx, Nasal Sinuses
• Minority show (+) M protein
• Progression to Multiple Myeloma
– Common in solitary Osseous myeloma
( 10-20 yrs)
– Less common in Extraosseous
55. EXTRANODAL NK/T-CELL
LYMPHOMA
• PREVIOUSLY MIDLINE GRANULOMA
• 3% OF NHL IN ASIA
• DESTRUCTIVE MIDLINE MASS
INVADE SMALL VESSELS
EXTENSIVE ISCHEMIC NECROSIS
• NEOPLASTIC ELEMENTS
– MIXTURE OF SMALL & LARGE LYMPHOID
CELLS
