clasificacion de linfomas b
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CLASIFICACION DE LINFOMAS
DRA. M. PILAR PAREDES MANRIQUE
HNGAI
Clasificación de la OMS
Neoplasias linfoides son neoplasias clonales de células inmaduras o maduras:– Linfoides B– Linfoides T– Linfoides NK
Tienden a recapitular de estadios normales de diferenciación 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 linfoblástico B, no especificado de otro modo– Leucemia/linfoma linfoblástico B con alteraciones genéticas
recurrentes– Leucemia/linfoma linfoblástico T
Neoplasias de células B maduras Neoplasias de células 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 - Burkitt’s 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
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
WHO classification
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 curablePrognosis: responsive to intensive combination chemoRx
better prognosis: limited Dz, small lesionbcl 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% High risk (4-5 points) - 5-year survival of 26%
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 lymphoma
2. sporadic(nonendemic) Burkitt lymphoma
3. HIV-associated neoplasm
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 LymphomaImmunophenotype:
sIgM, light chainCD19, CD20, CD10CD5 -, CD23 -
Genetics:translocation of c-myc gene
t(8;14)t(2;8)t(8;22)
EBV infection100% of African Burkitt25% 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 lymphocytes
-absence of centroblast-like cells
nucleus: round to irregular occasionally
cleaved condensed
chromatic inconspicuous
nucleoliscant cytoplasm
Mantle Cell Lymphoma
Immunophenotype:
Pos: CD19, CD20, CD5(aberrant), CD43
sIgM, sIgD
light chain (k or l)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 40’s, 50’s 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
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