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B Cell Lymphoma, Unclassifiable, Transformed from Follicular Lymphoma: A Rare Presentation with Review of the Literature.

Kanna A, Agrawal S, Jayant K, Kumar Pala V, Altujjar M, Hadid T, Khurram M - Case Rep Hematol (2015)

Bottom Line: He was diagnosed with BCLu-DLBCL/CHL.The patient received "ACVBP" (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation.BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression.

View Article: PubMed Central - PubMed

Affiliation: Guntur Medical College, Andhra Pradesh 522004, India.

ABSTRACT
B cell lymphoma, unclassifiable, with features of diffuse large B cell lymphoma and classical Hodgkin's lymphoma (BCLu-DLBCL/CHL) is more commonly known as gray zone lymphoma. These cases more often present with mediastinal disease. In this report, we present a very rare case of BCLu-DLBCL/CHL without mediastinal involvement, transformed from follicular lymphoma (FL) to BCLu-DLBCL/CHL. This patient initially presented with a mass in the right neck; biopsy of the lymph node showed predominantly nodular, follicular pattern. Immunohistochemical (IHC) staining of tumor cells expressed positivity for mature B cell markers CD20, CD19, CD10, CD23, CD45, and CD38 but negative for CD5,11c. Hence, diagnosed with FL, he was given rituximab, cyclophosphamide, vincristine, and prednisone (RCVP) regimen, followed by maintenance rituximab. He showed good response. After 2 years, he presented again with a mass in the right side of the neck. Although the needle core biopsy of this mass was suggestive of B cell lymphoma, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. IHC staining expressed positivity for CD20, CD79a, PAX5, and CD15 and CD30 consistent with DLBCL and CHL. He was diagnosed with BCLu-DLBCL/CHL. The patient received "ACVBP" (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation. BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression.

No MeSH data available.


Related in: MedlinePlus

CD 79a positivity suggestive of DLCBL (100x).
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Related In: Results  -  Collection


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fig7: CD 79a positivity suggestive of DLCBL (100x).

Mentions: After 2 years of follow-up period, he presented again with a mass on the right side of the neck, asymptomatic. Left cervical lymphadenopathy was also present. FDG/PET-CT scan showed multiple hypermetabolic lymph nodes with SUV max uptake of 15.6, measuring 4.4 × 1.9 cm consistent with recurrent lymphoma (Figure 4). Laboratory examination revealed WBC count of 6100/mcL (normal range 3600–11000/mcL), hemoglobin of 15.3 gm/dL (normal range 13.0–16.0 gm/dL), platelets of 1,22,000/mcL (normal range 130000–450000/mcL) and LDH of 155 U/L (normal range 135–225 U/L). Needle core biopsy of the LN on the right side of the neck showed findings suggestive of large B cell lymphoma. However, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. Several large cells were noticed, of which many were binucleated and multinucleated. Prominent mitotic activity and pleomorphism were noted (Figures 5(a), 5(b), and 5(c)). Immunohistochemical staining of the tumor cells showed positivity for CD 20 (Figure 6), CD79a (Figure 7), PAX 5 (Figure 8), bcl-2, and bcl-6+ consistent with DLBCL and CD 15 (Figure 9) and CD 30 (Figure 10) consistent with CHL and negativity for CD3, CD5, CD10, ALK-1 protein, EMA, and Epstein Barr Virus (EBV) (EBER by ISH-EBV- (Epstein-Bar Virus-) Encoded RNA (Ribonucleic Acid) In Situ Hybridization). Ki-67 was extremely high with approximately 80% (Figure 11). CD 45 was equivocal; however, in foci, there was a suggestion of foci of weak positivity. CD 21 highlighted the residual follicular dendritic meshwork of the residual follicles in the lymph node. The histologic pattern shows foci with an appearance of classical Hodgkin's lymphoma, as well as areas with morphological features of a DLBCL. With the histologic features and pattern, as well as the immunohistochemical staining pattern, the findings were more consistent with BCLu-DLBCL/CHL. This tumor showed tumor transformation from follicular lymphoma to BCLu-DLBCL/CHL, which is a very rare condition. Flow cytometry analysis from excisional biopsy showed no evidence of monoclonal B cell population or aberrant T cell antigen expression immunophenotypically. Bone marrow biopsy was negative. Bone marrow cytogenetic analysis noted normal male karyotype 46XY.


B Cell Lymphoma, Unclassifiable, Transformed from Follicular Lymphoma: A Rare Presentation with Review of the Literature.

Kanna A, Agrawal S, Jayant K, Kumar Pala V, Altujjar M, Hadid T, Khurram M - Case Rep Hematol (2015)

CD 79a positivity suggestive of DLCBL (100x).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4563079&req=5

fig7: CD 79a positivity suggestive of DLCBL (100x).
Mentions: After 2 years of follow-up period, he presented again with a mass on the right side of the neck, asymptomatic. Left cervical lymphadenopathy was also present. FDG/PET-CT scan showed multiple hypermetabolic lymph nodes with SUV max uptake of 15.6, measuring 4.4 × 1.9 cm consistent with recurrent lymphoma (Figure 4). Laboratory examination revealed WBC count of 6100/mcL (normal range 3600–11000/mcL), hemoglobin of 15.3 gm/dL (normal range 13.0–16.0 gm/dL), platelets of 1,22,000/mcL (normal range 130000–450000/mcL) and LDH of 155 U/L (normal range 135–225 U/L). Needle core biopsy of the LN on the right side of the neck showed findings suggestive of large B cell lymphoma. However, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. Several large cells were noticed, of which many were binucleated and multinucleated. Prominent mitotic activity and pleomorphism were noted (Figures 5(a), 5(b), and 5(c)). Immunohistochemical staining of the tumor cells showed positivity for CD 20 (Figure 6), CD79a (Figure 7), PAX 5 (Figure 8), bcl-2, and bcl-6+ consistent with DLBCL and CD 15 (Figure 9) and CD 30 (Figure 10) consistent with CHL and negativity for CD3, CD5, CD10, ALK-1 protein, EMA, and Epstein Barr Virus (EBV) (EBER by ISH-EBV- (Epstein-Bar Virus-) Encoded RNA (Ribonucleic Acid) In Situ Hybridization). Ki-67 was extremely high with approximately 80% (Figure 11). CD 45 was equivocal; however, in foci, there was a suggestion of foci of weak positivity. CD 21 highlighted the residual follicular dendritic meshwork of the residual follicles in the lymph node. The histologic pattern shows foci with an appearance of classical Hodgkin's lymphoma, as well as areas with morphological features of a DLBCL. With the histologic features and pattern, as well as the immunohistochemical staining pattern, the findings were more consistent with BCLu-DLBCL/CHL. This tumor showed tumor transformation from follicular lymphoma to BCLu-DLBCL/CHL, which is a very rare condition. Flow cytometry analysis from excisional biopsy showed no evidence of monoclonal B cell population or aberrant T cell antigen expression immunophenotypically. Bone marrow biopsy was negative. Bone marrow cytogenetic analysis noted normal male karyotype 46XY.

Bottom Line: He was diagnosed with BCLu-DLBCL/CHL.The patient received "ACVBP" (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation.BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression.

View Article: PubMed Central - PubMed

Affiliation: Guntur Medical College, Andhra Pradesh 522004, India.

ABSTRACT
B cell lymphoma, unclassifiable, with features of diffuse large B cell lymphoma and classical Hodgkin's lymphoma (BCLu-DLBCL/CHL) is more commonly known as gray zone lymphoma. These cases more often present with mediastinal disease. In this report, we present a very rare case of BCLu-DLBCL/CHL without mediastinal involvement, transformed from follicular lymphoma (FL) to BCLu-DLBCL/CHL. This patient initially presented with a mass in the right neck; biopsy of the lymph node showed predominantly nodular, follicular pattern. Immunohistochemical (IHC) staining of tumor cells expressed positivity for mature B cell markers CD20, CD19, CD10, CD23, CD45, and CD38 but negative for CD5,11c. Hence, diagnosed with FL, he was given rituximab, cyclophosphamide, vincristine, and prednisone (RCVP) regimen, followed by maintenance rituximab. He showed good response. After 2 years, he presented again with a mass in the right side of the neck. Although the needle core biopsy of this mass was suggestive of B cell lymphoma, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. IHC staining expressed positivity for CD20, CD79a, PAX5, and CD15 and CD30 consistent with DLBCL and CHL. He was diagnosed with BCLu-DLBCL/CHL. The patient received "ACVBP" (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation. BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression.

No MeSH data available.


Related in: MedlinePlus