CLINICAL HISTORY
53/F presented with multiple enlarged cervical lymph nodes. Biopsy of the lymph node shown in images. Immunohistochemical/flowcytometric analysis revealed the following phenotype:
ANTIBODY
RESULT
COMMENTS
CD3
Negative
CD20
Positive
Dim
CD10/CALLA
Positive
Dim
CD30
Negative
BCL-2
Positive
Diffuse strong
BCL-6
Positive
Focal
Cyclin-D1
Negative
Ki-67:MIB-1
>80%
MUM1/IRF4
Positive
CD138 (Syndecan-1)
Negative
CD45:LCA
Positive
CD79a
Positive
PAX-5
Positive
Epithelial Membrane Antigen
Negative
CK-AE1/3 Cocktail
Negative
CAM5.2
Negative
MOLECULAR GENETICS SHOWED:
PROBE
# NUCLEI EXAMINED
% POSITIVE NUCLEI
% CONTROL
8q24(MYC)
200
77.0
0-3.8
IGH/MYC/CEP8 t(8;14)
200
81.0
0-3.4
PHOTOMICROGRAPHS:
Figures 1 & 2: Low power views of lymph node showing effacement of architecture with diffuse and follicular infiltrate of atypical lymphoid cells.
Figures 3 & 4: Medium and high power views of lymph node showing a monotonous population of medium to large sized cells with round to oval nuclei, 1-3 prominent nucleoli and scanty blue cytoplasm. There are numerous mitoses and apoptotic activity.
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Question 1 of 4
1. Question
What is your diagnosis?
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Aggressive B-cell lymphomas are clinically and pathologically diverse. MYC translocations are commonly associated with Burkitt lymphoma, but deregulations of MYC are shown to occur in other aggressive lymphomas as well, mainly as a ‘second-hit’. The double-hit lymphomas with MYC and either BCL2 or BCl6 are considered very aggressive and are included under the category B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL). These intermediate lymphomas exhibit a very high proliferation index similar to Burkitt lymphoma and may or may not show a starry sky pattern. Furthermore, recent evidences suggest that of the two entities, the double-hit lymphoma with MYC and BCL6 rearrangements are more aggressive than the one with MYC and BCL2. Thus distinguishing these entities from each other is important task in clinical practice.
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Aggressive B-cell lymphomas are clinically and pathologically diverse. MYC translocations are commonly associated with Burkitt lymphoma, but deregulations of MYC are shown to occur in other aggressive lymphomas as well, mainly as a ‘second-hit’. The double-hit lymphomas with MYC and either BCL2 or BCl6 are considered very aggressive and are included under the category B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL). These intermediate lymphomas exhibit a very high proliferation index similar to Burkitt lymphoma and may or may not show a starry sky pattern. Furthermore, recent evidences suggest that of the two entities, the double-hit lymphoma with MYC and BCL6 rearrangements are more aggressive than the one with MYC and BCL2. Thus distinguishing these entities from each other is important task in clinical practice.
Question 2 of 4
2. Question
What is the characteristic translocation described in Burkitt’s lymphoma?