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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.T cell leukemia-lymphoma HTLV-V .cutaneous T cell lymphoma Hepatitis C . adrenal LNH)  Viral causes     EBV .Burkitt’s lymphoma HTLV-I .

B2 microglobulina  Bone marrow biopsy  Imaging . R/L .FBC.CT N/T/A/P .PET/CT scan . uric acid .Diagnosis  Blood tests .CXR .LDH.

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

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

Immunodeficiency-associated large B-cell lymphoma -with latent EBV infection 2.Diffuse Large B-cell Lymphoma Special Subtypes 1. 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 .

5-year survival of 73%  Low-intermediate risk (2 points) .5-year survival of 51%  High-intermediate risk (3 points) . or 4 More than 1 extranodal site The sum of the points allotted correlates with the following risk groups:  Low risk (0-1 points) .International Prognostic Index      Age greater than 60 years Stage III or IV disease Elevated serum LDH ECOG/Zubrod performance status of 2.5-year survival of 43% . 3.

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) .

22) ----.preB-cell type t(9.Acute Lymphoblastic Leukemia/Lymphoma (ALL) Immunophenotype: TdT(+) --.>95% of cases Genetics: t(12.3% of children. 25% of adults t(4.21) --.11) hyperdiploidy pseudodiploidy .

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

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

peritoneum Prognosis: generally aggressive tumor. adrenal)  nonendemic Burkitt: ileocecum.Burkitt Lymphoma Clinical: children and young adults frequent extranodal involvent  African Burkitt: mandible abdominal organs (kidneys. but responds well to therapy . ovaries.

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

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. CD20.70% of cases involving bcl 1 gene  loss of cell cycle control .14) (elevated cyclin D1) -. CD23 ( B-CLL/SLL) Genetics:t(11. CD5(aberrant). sIgD light chain ( or ) Neg: CD10. CD43 sIgM.

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 .Mantle Cell Lymphoma Clinical:-males in 40’s. 50’s and older -generalized lymphadenopathy (widespread) -BM: para.