|Ahead of print publication
Transformation of diffuse large B-cell lymphoma to lymphoblastic lymphoma
Pritika Kushwaha1, Meeta Singh1, Varuna Mallya1, Shyama Jain1, Sunita Aggarwal2, Kishore Singh3
1 Department of Pathology, Maulana Azad Medical College, New Delhi, India
2 Department of Medicine, Maulana Azad Medical College, New Delhi, India
3 Department of Radiotherapy, Maulana Azad Medical College, New Delhi, India
|Date of Submission||11-Jan-2021|
|Date of Acceptance||30-Apr-2021|
|Date of Web Publication||22-Nov-2021|
Department of Pathology, Maulana Azad Medical College, New Delhi
Source of Support: None, Conflict of Interest: None
Transformations in diffuse large B-cell lymphoma (DLBCL) are extremely rare. Here, we are presenting a very rare case of DLBCL transforming into lymphoblastic lymphoma (LBL) diagnosed by fine-needle aspiration cytology (FNAC) and flow cytometry. A 31-year old male on antiretroviral therapy and a known case of diffuse large B-cell lymphoma diagnosed 1 year back on cervical lymphadenopathy, presented with left axillary swelling for 3 months. FNAC and Flow cytometry were performed from the left axillary swelling which confirmed the diagnosis of LBL.
Keywords: Diffuse large B-Cell Lymphoma, flow cytometry, fine-needle aspiration cytology, lymphoblastic lymphoma, transformation
| > Introduction|| |
Diffuse large B-cell lymphoma (DLBCL) is a high-grade lymphoma and the most common type of non-Hodgkin lymphoma (NHL) worldwide, representing approximately 30%–40% of adult NHL.
Transformations in DLBCLs are extremely rare with only scant literature available on transformation of DLBCL to lymphoblastic lymphoma (LBL).
Here, we are presenting a very rare case of DLBCL transforming into LBL diagnosed by fine-needle aspiration cytology (FNAC) and flow cytometry.
| > Case Report|| |
A 31-year-old male on antiretroviral therapy presented with cervical lymphadenopathy 1 year back. Excision biopsy of cervical lymph node was done and was diagnosed as DLBCL (CD 19+, CD20+, CD 10-, MUM 1+, and BCL 6-) [Figure 1]. He was started on chemotherapy, RCHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone). Having received three cycles of chemotherapy, the patient now presented with left axillary swelling for 3 months. On examination, 6 cm × 6 cm well-defined, non-tender swelling was palpable. He was referred for FNAC of the swelling.
|Figure 1: (a) Biopsy showed monomorphic population of large cells with high N:C ratio, prominent nucleoli (H and E, ×400). (b and c) Immunohistochemistry with DAB as chromogen (×600) – These cells showed strong expression of CD20 (b) and MUM1 (c)|
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FNAC was performed from the left axillary swelling using 23G needle attached with disposable 20 ml syringe, under aseptic precautions as per the standard technique. FNAC yielded blood mixed aspirate, multiple air-dried smears were prepared and stained with Giemsa. Cell block was prepared and stained with H and E.
Giemsa-stained smears showed large lymphoid cells with high N: C ratio and moderate amount of cytoplasm, some of the cells showed prominent nucleoli with blastoid morphology [Figure 2]. Complete blood count showed pancytopenia with the absence of blasts.
|Figure 2: Fine-needle aspiration cytology showed large lymphoid cells with high N:C ratio and moderate amount of cytoplasm, some of the cells showed prominent nucleoli with blastoid morphology (arrow) in hemorrhagic background (MGG, ×600)|
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Sample for flow cytometry was also obtained during FNAC in a plastic vial containing 1 ml sheath fluid. Flow cytometry immunophenotyping showed gated cells having moderate-to-high forward scatter and positivity for CD45, CD19, CD20, HLADR, CD34, Tdt, and kappa restriction [Figure 3]. These gated cells did not express CD3, CD4, CD7, CD8, CD38, CD56, and lambda confirmed on Trucut biopsy.
|Figure 3: Flow cytometry immunophenotyping showing cells expressing CD45, CD19, CD20, HLADR, and CD34 and showing kappa restriction|
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Based on the cytological and flow cytometry findings and the patient being a known case of DLBCL, diagnosis of transformation of DLBCL to LBL was rendered.
The patient succumbed to the illness few days after the diagnosis, no bone marrow could be performed or treatment could be started.
| > Discussion|| |
The most common histological transformation in malignant lymphoproliferative diseases is the high-grade transformation also known as Ritcher's transformation of chronic lymphocytic leukemia into DLBCL, plasmacytoma, acute lymphoblastic leukemia (ALL), or histiocytic sarcoma. The transformation of follicular lymphoma (FL), marginal zone lymphoma, and lymphoplasmacytoid lymphoma into DLBCL is also well recognized.
It has been reported that patients with HIV infection and autoimmune diseases show abnormal B-cell populations due to immunosuppression or antigen overexposure, both in circulation and in affected organs which may transform into clonal cells under antigen drive, ultimately leading to independent B-cell neoplasms to develop.,
Transformation of FL and mantle cell lymphoma into a B-LBL/leukemia occurs rarely and has been documented for a limited number of cases.,
However, transformations in DLBCL are extremely rare and on extensive literature search, single case of transformation of DLBCL to LBL has been reported, in which lymphoblastic transformation from a triple-hit DLBCL occurred along with occasional case reports of transformation of DLBCL to lymphoblastic leukemia.,
Transformation of DLBCL to LBL confers very poor prognosis. These cases often lead to extensive peripheral blood and bone marrow involvement. Treatment of such cases is initiated according to the ALL protocol.
Clinical, morphologic, cytogenetic, and immunophenotypic evaluations are necessary to fully characterize this type of transformation in lymphoma. FNAC with flow cytometry is a useful adjunct to early diagnosis.
| > Conclusion|| |
Clinical, morphologic, and immunophenotypic evaluations are necessary to fully characterize transformation of DLBCL to Lymphoblastic Lymphoma. FNAC with flow cytometry is a useful adjunct to its early diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]