CLASIFICACION DE

LINFOMAS
DRA. M. PILAR PAREDES MANRIQUE
HNGAI

Clasificacin de la OMS
Neoplasias

linfoides son neoplasias clonales de clulas

inmaduras o maduras:

Linfoides B
Linfoides T
Linfoides NK

Tienden

a recapitular de estadios normales de diferenciacin

B, T o NK.

Classification of Lymphoma
Histopathological

NHL vs HL
B vs T-cell
CD20 +ve vs CD20 -ve
high grade vs low grade

Clinical

aggressive vs indolent
stage I vs IV

Molecular

c-myc gene translocation

Neoplasias linfoides OMS 2008

Neoplasias

de precursores linfoides:

Leucemia/linfoma linfoblstico B, no especificado de otro modo

Leucemia/linfoma linfoblstico B con alteraciones genticas
recurrentes
Leucemia/linfoma linfoblstico T

Neoplasias

de clulas B maduras
Neoplasias de clulas T y NK maduras

Etiology
The exact etiology is unknown
Immune suppression

congenital (Wiskott-Aldrich syndrome)

organ transplant (immunosupressants)
HIV infection
increasing age

DNA repair defects

ataxia telangiectasia
xeroderma pigmentosum

Etiology
Chronic inflammation

Helicobacter pylori (gastric NHL)

Chlamydia psittaci (ocular, adrenal LNH)

Viral causes

EBV - Burkitts lymphoma

HTLV-I - T cell leukemia-lymphoma
HTLV-V - cutaneous T cell lymphoma
Hepatitis C

Diagnosis
Blood tests
- FBC, R/L
- LDH, uric acid
- B2 microglobulina

Bone marrow biopsy

Imaging
- CXR
- CT N/T/A/P
- PET/CT scan

WHO classification

B cell lymphoma

lymphoblastic leukemia/lymphoma
follicular lymphoma
chronic lymphocytic leukemia/small lymphocytic lymphoma
mantle cell lymphoma
prolymphocytic leukemia
hairy cell leukemia
lymphoplasmacytic lymphoma
marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT)-type
nodal marginal zone B cell lymphoma
splenic marginal zone B cell lymphoma
Burkitt lymphoma
diffuse large B cell lymphoma
plasmacytoma
plasma cell myeloma

Diffuse Large B-cell Lymphoma

Overview: -heterogeneous peripheral B-cell tumor
-20% of all NHL, 60-70% of aggressive lymphoid tumors
Morphology:
-diffuse growth pattern
-large tumor cells

Diffuse Large B-cell Lymphoma

Immunophenotype:
Pos:CD19, CD20, CD22, CD79a, (CD10), sIg
Neg: TdT
Genetics:
t(14;18)(= bcl-2 rearrangement)-- 30% of cases
--- 20 to 30% of cases bcl 6 rearrangement (CH3)
extranodal lesions: negative for bcl 2 rearrangement

Diffuse Large B-cell Lymphoma

Clinical: -wide age range (median age : 60 y/o)
-slight male predominance
-rapidly enlarging mass
(GI tract, skin, bone, brain,
ORL
lymphoid tissue, liver, spleen)
-aggressive behavior, but potentially curable
Prognosis: responsive to intensive combination chemoRx
better prognosis: limited Dz, small lesion
bcl 6
rearrangement
worse prognosis: p53 mutation

Diffuse Large B-cell Lymphoma

Special Subtypes
1. Immunodeficiency-associated large B-cell lymphoma
-with latent EBV infection
2. body cavity large B-cell lymphoma
-mostly seen in advanced HIV(+) patients
-arises as malignant pleural or ascitic effusion
-infected with human herpes virus 8

International Prognostic
Index

Age greater than 60 years

Stage III or IV disease
Elevated serum LDH
ECOG/Zubrod performance status of 2, 3, or 4
More than 1 extranodal site

The sum of the points allotted correlates with the following

risk groups:
Low risk (0-1 points) - 5-year survival of 73%
Low-intermediate risk (2 points) - 5-year survival of 51%
High-intermediate risk (3 points) - 5-year survival of 43%

Acute Lymphoblastic
Leukemia/Lymphoma (ALL)
Overview: B-cell tumor (85%)
T-cell tumors tend to be seen in adolescent
males with frequent thymic involvement
differential diagnosis from AML important
Morphology:PAS(+) cytoplasmic granules
absence of peroxidase(+) granules ( AML)

Acute Lymphoblastic
Leukemia/Lymphoma (ALL)
Immunophenotype: TdT(+) --- >95% of cases
Genetics: t(12;21) --- preB-cell type
t(9;22) ----- 3% of children, 25% of adults
t(4;11)
hyperdiploidy
pseudodiploidy

Burkitt Lymphoma
Overview: relatively mature B-cell tumor
three(3) subtypes:
1. African(endemic) Burkitt
2. sporadic(nonendemic)
lymphoma
3. HIV-associated neoplasm

lymphoma
Burkitt

Burkitt Lymphoma
Morphology:

diffuse infiltrate of
intermediate-size tumor
cells

round to oval nucleus

coarse chromatin
several nucleoli
moderate amount of
basophilic cytoplasm with
vacuoles

high mitotic rate

apoptotic tumor cell death
numerous macrophages
(starry sky appearance)

Burkitt Lymphoma
Immunophenotype:
sIgM, light chain
CD19, CD20, CD10
CD5 -, CD23 Genetics:
translocation of c-myc gene
t(8;14)
t(2;8)
t(8;22)
EBV infection
100% of African Burkitt
25% of HIV-associated Burkitt

Burkitt Lymphoma
Clinical:
children and young adults
frequent extranodal involvent
African Burkitt: mandible
abdominal organs (kidneys, ovaries, adrenal)
nonendemic Burkitt: ileocecum, peritoneum
Prognosis:

generally aggressive tumor, but responds well to

therapy

Mantle Cell Lymphoma

Overview: peripheral B-cell tumor
3% of NHL in U.S. (7-9% in Europe)
Morphology: two patterns of LN involvement
1. mantle zone pattern
2. diffuse pattern

Normal

Mantle Cell Lymphoma

Morphology:-homogeneous population of small
-absence of centroblast-like cells

lymphocytes

nucleus: round to irregular

occasionally cleaved
condensed chromatic
inconspicuous nucleoli
scant cytoplasm

Mantle Cell Lymphoma

Immunophenotype:
Pos: CD19, CD20, CD5(aberrant), CD43
sIgM, sIgD
light chain ( or )
Neg: CD10, CD23 ( B-CLL/SLL)
Genetics:t(11;14) (elevated cyclin D1) -- 70% of cases
involving bcl 1 gene loss of cell cycle control

Mantle Cell Lymphoma

Clinical:-males in 40s, 50s and older
-generalized lymphadenopathy (widespread)
-BM: para- and non-trabecular aggregates
-extranodal involvement including liver & spleen
lymphomatoid polyposis
multifocal entero-colic mucosal involvement
Prognosis: poor (median survival : 3-5 yrs)
not curable with conventional chemotherapy
death due to organ dysfunction