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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

Flow cytometry of the bone marrow biopsy sample, negative.
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Related In: Results  -  Collection


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fig1: Flow cytometry of the bone marrow biopsy sample, negative.

Mentions: A 56-year-old male presented with mass in the right neck area, asymptomatic at the time of presentation. Biopsy of the lymph node (LN) in the right side of the neck was done. Sections of the lymph node reveal replacement of the normal architecture with a predominantly nodular, follicular pattern. About 10% of the lymph node shows more diffuse proliferation. The follicles showed a mixture of small lymphocytes, larger centroblasts, and scattered follicular dendritic cells, consistent with follicular lymphoma grade 2 of 3. Flow cytometry showed a monoclonal B cell population with kappa cells. Tumor cells, on immunohistochemical staining, expressed positivity for mature B cell markers CD-20, CD-19, CD-10, CD-23, CD-45, and CD-38 but were negative for CD-5,11c. Therefore, he was diagnosed with follicular lymphoma. The bone marrow biopsy was normal cellularity with no evidence of involvement. Flow cytometry results showed no evidence of monoclonal B cell population or an immunophenotypically aberrant T cell population (Figure 1). Cytogenetic study (Figure 2) and FISH analysis for t(14;18) (Figure 3) did not reveal any abnormalities in the bone marrow biopsy sample. Computerised Tomography (CT) scan showed disease both above and below the diaphragm and the largest lymph node was under 3 cms. Hence, he was diagnosed with follicular lymphoma grade 2, stage III. He was placed under observation. In due course, after 1 year, he developed renal insufficiency with an increase in his serum creatinine to 1.7 mg/dL (normal range: 0.6–1.4 mg/dL). CT scan showed enlarged retrocaval LN obstructing left ureter causing left kidney hydronephrosis. Palliative ureteral stenting was done. He was started on chemotherapy after 6 months. He received a total of 6 courses of RCVP (rituximab 375 mg/m2 IV on day 1 plus cyclophosphamide 750 mg/m2 IV on day 1 plus vincristine 1.4 mg/m2 (dose cap at 2 mg) IV on day 1, plus prednisone 40 mg/m2 PO on days 1–5; every 21 days for six cycles) and maintenance rituximab was given as per the Hainsworth protocol, that is, four-weekly rituximab every 6 months. Rituximab was given 4 times, as maintenance. After his induction regimen of RCVP, he was followed with Fluorodeoxyglucose positron emission tomography- (FDG/PET-) CT scan every month which showed residual uptake in the right retroperitoneal area on the initial follow-up FDG/PET-CT scan. In the due course, the standardized uptake value (SUV) gradually declined from 5.4 to 0. Also, this patient recovered from the left hydronephrosis and the stent was removed, though underlying chronic renal insufficiency still persisted. Since the completion of rituximab, he has been under surveillance. He responded well to the treatment given and was in remission.


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)

Flow cytometry of the bone marrow biopsy sample, negative.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig1: Flow cytometry of the bone marrow biopsy sample, negative.
Mentions: A 56-year-old male presented with mass in the right neck area, asymptomatic at the time of presentation. Biopsy of the lymph node (LN) in the right side of the neck was done. Sections of the lymph node reveal replacement of the normal architecture with a predominantly nodular, follicular pattern. About 10% of the lymph node shows more diffuse proliferation. The follicles showed a mixture of small lymphocytes, larger centroblasts, and scattered follicular dendritic cells, consistent with follicular lymphoma grade 2 of 3. Flow cytometry showed a monoclonal B cell population with kappa cells. Tumor cells, on immunohistochemical staining, expressed positivity for mature B cell markers CD-20, CD-19, CD-10, CD-23, CD-45, and CD-38 but were negative for CD-5,11c. Therefore, he was diagnosed with follicular lymphoma. The bone marrow biopsy was normal cellularity with no evidence of involvement. Flow cytometry results showed no evidence of monoclonal B cell population or an immunophenotypically aberrant T cell population (Figure 1). Cytogenetic study (Figure 2) and FISH analysis for t(14;18) (Figure 3) did not reveal any abnormalities in the bone marrow biopsy sample. Computerised Tomography (CT) scan showed disease both above and below the diaphragm and the largest lymph node was under 3 cms. Hence, he was diagnosed with follicular lymphoma grade 2, stage III. He was placed under observation. In due course, after 1 year, he developed renal insufficiency with an increase in his serum creatinine to 1.7 mg/dL (normal range: 0.6–1.4 mg/dL). CT scan showed enlarged retrocaval LN obstructing left ureter causing left kidney hydronephrosis. Palliative ureteral stenting was done. He was started on chemotherapy after 6 months. He received a total of 6 courses of RCVP (rituximab 375 mg/m2 IV on day 1 plus cyclophosphamide 750 mg/m2 IV on day 1 plus vincristine 1.4 mg/m2 (dose cap at 2 mg) IV on day 1, plus prednisone 40 mg/m2 PO on days 1–5; every 21 days for six cycles) and maintenance rituximab was given as per the Hainsworth protocol, that is, four-weekly rituximab every 6 months. Rituximab was given 4 times, as maintenance. After his induction regimen of RCVP, he was followed with Fluorodeoxyglucose positron emission tomography- (FDG/PET-) CT scan every month which showed residual uptake in the right retroperitoneal area on the initial follow-up FDG/PET-CT scan. In the due course, the standardized uptake value (SUV) gradually declined from 5.4 to 0. Also, this patient recovered from the left hydronephrosis and the stent was removed, though underlying chronic renal insufficiency still persisted. Since the completion of rituximab, he has been under surveillance. He responded well to the treatment given and was in remission.

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