|Year : 2015 | Volume
| Issue : 1 | Page : 54-56
Non-Hodgkin lymphoma unclassifiable between diffuse large B-cell lymphoma and Burkitt lymphoma
Ashok Singh1, Suvendu Purkait1, Ajay Gogia2, Saumyaranjan Mallick1
1 Department of Pathology, All Institute of Medical Sciences, New Delhi, India
2 Department of Medical Oncology, All Institute of Medical Sciences, New Delhi, India
|Date of Submission||30-Aug-2015|
|Date of Acceptance||07-Oct-2015|
|Date of Web Publication||9-Nov-2015|
Department of Pathology, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Large B-cell lymphomas are the most common non-Hodgkin lymphoma representing a heterogeneous group of diseases which often pose diagnostic problems in the clinical practice. B-cell lymphoma, unclassifiable (B-UCL), with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma, is a poorly defined rare entity. We describe a 59-year-old male patient presented with complaints of bloody nasal discharge for last few months. On examination, a nasal mass was seen. Biopsy revealed a non-Hodgkin lymphoma. The cells are immune positive for CD20, BCL-2, BCL-6, and MUM-1. Ki-67 labeling index was 90% while negative for CD1 and CD5. A diagnosis of B-UCL was made. This case is presented due to its diagnostic difficulty among large B-cell lymphomas and emphasizes the need to integrate morphological and immunohistochemical findings.
Keywords: Burkitt lymphoma, non Hodgekins lymphoma, large cell lymphoma
|How to cite this article:|
Singh A, Purkait S, Gogia A, Mallick S. Non-Hodgkin lymphoma unclassifiable between diffuse large B-cell lymphoma and Burkitt lymphoma. J Curr Res Sci Med 2015;1:54-6
|How to cite this URL:|
Singh A, Purkait S, Gogia A, Mallick S. Non-Hodgkin lymphoma unclassifiable between diffuse large B-cell lymphoma and Burkitt lymphoma. J Curr Res Sci Med [serial online] 2015 [cited 2021 Jul 30];1:54-6. Available from: https://www.jcrsmed.org/text.asp?2015/1/1/54/168920
| Introduction|| |
Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma accounts for around 20–40% cases. Due to the diverse clinical behavior and genetic profile, World Health Organization (WHO) (2008) describes various subtypes. Non-Hodgkin lymphoma having ambiguity in immunohistochemical profile and morphological findings have been classified by WHO in separate entities owing to different biological behavior as compared to their well-established counterparts in which they do not fit (WHO). Diagnosing these cases is difficult and requires a thorough molecular workup and morphological and immunohistochemical entities. In this border line category, two main groups are gray zone and B-cell lymphoma, unclassifiable (B-UCL) with features intermediate between DLBCL and Burkitt lymphoma. On the other hand, diagnosing them is important as there are no effective treatment protocols for these entities.
| Case History|| |
A 59-year-old male patient presented to the Otorhinology Out-patient Department with complaints of bloody nasal discharge for last few months. Clinical examination revealed absence of pallor, jaundice, cyanosis, clubbing, subcutaneous nodules, or lymphadenopathy. On examination, a nasal mass was seen. The vital parameters were within normal limits. Baseline complete blood count revealed the following: Hemoglobin 9.4 g/dl; white blood cell count, 5.83 × 109/L; platelet count, 2 × 109/L; and occasional atypical lymphocytes per 100 white blood cells; and serum lactate dehydrogenase was1357 U/L. Ultrasonography abdomen was normal. The nasal mass was excised and sent for histopathological examination.
Histopathology showed monotonous population of intermediate to large lymphoid cells with mild nuclear irregularities and pleomorphism. Few of the cells show multiple small nucleoli. Brisk mitotic activity and lymphoglandular bodies were also noted. Morphologically, diagnosis of large B-cell lymphoma was made. Immunohistochemistry showed the cells are positive for CD20, BCL-2, BCL-6, and MUM-1 while negative for CD3, CD5, TDT, and CD1. The Ki-67 labeling index was 90% [Figure 1]. The morphological picture was more of a Burkitt lymphoma. The immunopositivity of BCL-2 (strong and patchy) were against the diagnosis. After reviewing the literature, a diagnosis of B-UCL was given. Cytogenetics for C-MYC could not be performed due to its unavailability.
|Figure 1: Diffuse effacement of lymphnodal architecture (a, H and E, ×200). There is a diffuse infiltrate by medium to large sized cells with nuclear irregularities and single central, prominent nucleoli. Brisk mitotic activity Immunohistochemistry showed the atypical lymphocytes are positive for CD10, BCL-6, BCL-2 (strong and patchy), and MUM-1 and negative staining with TdT. Ki-67 labeling index is 90%|
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| Discussion|| |
B-UCL is now a separate entity recognized by WHO comprises of a non-Hodgkin lymphoma which is of high grade not fitting into any existing categories based on morphological and immunohistochemical features. These lymphomas are mostly missed and misdiagnosed as Burkitt lymphoma or DLBCL. The diagnosis of this entity is important as it has a poor response to established treatment protocols of Burkitt lymphoma and DLBCL., A high grade lymphoma with morphological features of Burkitt lymphoma but atypical immunophenotype or high grade lymphoma with typical immunophenotype of Burkitt lymphoma, but atypical morphology or having atypical morphological and immunophenotype not fitting into Burkitt lymphoma are being designated at B-UCL. This lymphoma mostly occur from the fourth to sixth decade with only occasional pediatric cases. Both nodal and extranodal presentation is reported.,, Bone marrow involvement is commonly seen and few patients also show leukemic phase. Morphologically, the individual cells are intermediate to large size with multiple nucleoli and have mild pleomorphism. Cytogenetic shows 8q24 and MYC translocation in 50% cases with aberrant cytogenetic abnormality with non-IG partner along with complex karyotype., These lymphomas generally have cytogenetic abnormality of BCL-2 and BCL-6 also including cases of double hit and triple hit lymphomas ,, Immunohistochemically, the lymphoma cells express CD20, BCL-2, and MYC. The Ki67 proliferating index was found to be around 90% unlike Burkitt lymphoma which shows a labelling index of 100%. In the present case, strong BCL-2 expression and a proliferation index below 95% excludes a diagnosis of BL. Few of the cases had a history of follicular lymphoma, so there may be the MYC/IG translocation that is secondary event after a prior BCL-2/IG translocation.
Chemokine's expression has also been reported. No consensus on treatment protocols as a patient does not respond to standard regimens for DLBCL and Burkitt lymphoma.,
| Conclusion|| |
A high-grade lymphoma showing features overlapping between DLBCL and Burkitt lymphoma should undergo thorough immunohistochemical and genetic testing before making a diagnosis of B-UCL with MYC mutation testing as these patients have a poorer prognosis.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al
. B-cell lymphoma unclassifiable, with features intermediate between diffuse large b-cell lymphoma and burkitt lymphoma. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2008.
Perry AM, Crockett D, Dave BJ, Althof P, Winkler L, Smith LM, et al.
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and burkitt lymphoma: Study of 39 cases. Br J Haematol 2013;162:40-9.
Klapper W, Szczepanowski M, Burkhardt B, Berger H, Rosolowski M, Bentink S, et al.
Molecular profiling of pediatric mature B-cell lymphoma treated in population-based prospective clinical trials. Blood 2008;112:1374-81.
Seo JY, Ha KY, Kim MU, Kim YC, Kim YH. Spinal cord compression by B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a patient seropositive for human immunodeficiency virus: A case report. J Med Case Rep 2014;8:324.
Nakayama S, Yokote T, Iwaki K, Hiraoka N, Hirata Y, Akioka T, et al.
Central nervous system infiltration of a multiple cytokine-producing double-hit B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma with CC chemokine receptor 7 expression. Acta Oncol 2013;52:1217-20.
Bellan C, Stefano L, Giulia de F, Rogena EA, Lorenzo L. Burkitt lymphoma versus diffuse large B-cell lymphoma: A practical approach. Hematol Oncol 2009;27:182-5.
Aukema SM, Siebert R, Schuuring E, van Imhoff GW, Kluin-Nelemans HC, Boerma EJ, et al.
Double-hit B-cell lymphomas. Blood 2011;117:2319-31.
Bellan C, Stefano L, Giulia de F, Rogena EA, Lorenzo L. Burkitt lymphoma versus diffuse large B-cell lymphoma: A practical approach. Hematol Oncol 2010;28:53-6.
Yano T, Jaffe ES, Longo DL, Raffeld M. MYC rearrangements in histologically progressed follicular lymphomas. Blood 1992;80:758-67.