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Unusual presentation of duodenal plasmablastic lymphoma in an immunocompetent patient: A case report and literature review.

Cao C, Liu T, Lou S, Liu W, Shen K, Xiang B - Oncol Lett (2014)

Bottom Line: To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient.Therefore, PBL represents a diagnostic and therapeutic challenge.PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing 400010, P.R. China ; Department of Hematology, West China Hospital, Sichuan University, Key Laboratory of Hematology of Sichuan Province, Chengdu, Sichuan 610041, P.R. China.

ABSTRACT
Plasmablastic lymphoma (PBL) is a rare and recently described entity of large B-cell lymphoma. It predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)-positive patients and exhibits a highly aggressive clinical behavior without effective treatment. Recently, sporadic cases describing PBL in extraoral locations of HIV-negative patients have been reported; frequently in patients with underlying immunosuppressive states. To develop the understanding of PBL, the current study reports the unusual presentation of duodenal PBL and reviews the pathogenesis, immunohistochemical features, clinical and differential diagnoses, as well as the treatment of PBL as described in previous studies. The case of a 75-year-old female with duodenal PBL without definite immunosuppression is presented in the current report. The tumor was composed of large B-cell-like cells, and was positive for cluster of differentiation 138 and melanoma ubiquitous mutated-1, with ~80% of the tumor cells positive for Ki-67. The features of the tumor were as follows: Extraoral location, HIV-negative, immunoglobulin M λ-type M protein expression, light chain restriction (monoclonal) and Epstein-Barr virus-encoded small RNA-negative, which are considered to be unusual for PBL. These unusual features complicate the differentiation of PBL from other plasma cell diseases. To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient. To date, the standard treatment of PBL remains elusive, however, the most commonly administered chemotherapy treatments are CHOP [intravenous cyclophosphamide (750 mg/m(2), day 1), intravenous doxorubicin (50 mg/m(2), day 1), intravenous vincristine (1.4 mg/m(2), day 1) and prednisone (100 mg, days 1-50)]-like regimens. The patient was administered two cycles of CHOP chemotherapy for 56 days, however, ultimately succumbed as a result of disease progression. Therefore, PBL represents a diagnostic and therapeutic challenge. PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients. Furthermore, CHOP does not appear to be an optimal treatment regimen and more intensive regimens are required.

No MeSH data available.


Related in: MedlinePlus

Immunohistochemical analysis of neoplastic cells (A) stained with hematoxylin and eosin, which were positive for (B) cluster of differentiation 138, (C) melanoma ubiquitous mutated-1, (D) Ki-67, (E) Epstein-Barr virus-encoded small RNA and (F) human herpesvirus 8.
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f2-ol-08-06-2539: Immunohistochemical analysis of neoplastic cells (A) stained with hematoxylin and eosin, which were positive for (B) cluster of differentiation 138, (C) melanoma ubiquitous mutated-1, (D) Ki-67, (E) Epstein-Barr virus-encoded small RNA and (F) human herpesvirus 8.

Mentions: The pathological results revealed the diffuse proliferation of neoplastic cells that exhibited a large B-like appearance, with a high proliferative index (Ki-67 of >80%) and frequent mitotic figures. Upon immunostaining, the neoplastic cells were positive for cluster of differentiation (CD) 138, melanoma ubiquitous mutated-1 (MUM-1), endomysial antibodies (EMA), IgM and Igλ; weakly positive for CD45 and CD10; and negative for CD19, CD20, CD3, CD5, CD79a, CD56, a B-cell-specific activator protein (PAX5), cyclin D1, granzyme B, B-cell lymphoma (BCL)-6, CD30, CEA, anaplastic lymphoma kinase (ALK), EBV-encoded small RNA (EBER) and HHV8 (Fig. 2). Monoclonal rearrangement of the immunoglobulin heavy chain gene and clonal restriction of the λ light chain gene expression were detected via polymerase chain reaction and heteroduplex analyses.


Unusual presentation of duodenal plasmablastic lymphoma in an immunocompetent patient: A case report and literature review.

Cao C, Liu T, Lou S, Liu W, Shen K, Xiang B - Oncol Lett (2014)

Immunohistochemical analysis of neoplastic cells (A) stained with hematoxylin and eosin, which were positive for (B) cluster of differentiation 138, (C) melanoma ubiquitous mutated-1, (D) Ki-67, (E) Epstein-Barr virus-encoded small RNA and (F) human herpesvirus 8.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4214469&req=5

f2-ol-08-06-2539: Immunohistochemical analysis of neoplastic cells (A) stained with hematoxylin and eosin, which were positive for (B) cluster of differentiation 138, (C) melanoma ubiquitous mutated-1, (D) Ki-67, (E) Epstein-Barr virus-encoded small RNA and (F) human herpesvirus 8.
Mentions: The pathological results revealed the diffuse proliferation of neoplastic cells that exhibited a large B-like appearance, with a high proliferative index (Ki-67 of >80%) and frequent mitotic figures. Upon immunostaining, the neoplastic cells were positive for cluster of differentiation (CD) 138, melanoma ubiquitous mutated-1 (MUM-1), endomysial antibodies (EMA), IgM and Igλ; weakly positive for CD45 and CD10; and negative for CD19, CD20, CD3, CD5, CD79a, CD56, a B-cell-specific activator protein (PAX5), cyclin D1, granzyme B, B-cell lymphoma (BCL)-6, CD30, CEA, anaplastic lymphoma kinase (ALK), EBV-encoded small RNA (EBER) and HHV8 (Fig. 2). Monoclonal rearrangement of the immunoglobulin heavy chain gene and clonal restriction of the λ light chain gene expression were detected via polymerase chain reaction and heteroduplex analyses.

Bottom Line: To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient.Therefore, PBL represents a diagnostic and therapeutic challenge.PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing 400010, P.R. China ; Department of Hematology, West China Hospital, Sichuan University, Key Laboratory of Hematology of Sichuan Province, Chengdu, Sichuan 610041, P.R. China.

ABSTRACT
Plasmablastic lymphoma (PBL) is a rare and recently described entity of large B-cell lymphoma. It predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)-positive patients and exhibits a highly aggressive clinical behavior without effective treatment. Recently, sporadic cases describing PBL in extraoral locations of HIV-negative patients have been reported; frequently in patients with underlying immunosuppressive states. To develop the understanding of PBL, the current study reports the unusual presentation of duodenal PBL and reviews the pathogenesis, immunohistochemical features, clinical and differential diagnoses, as well as the treatment of PBL as described in previous studies. The case of a 75-year-old female with duodenal PBL without definite immunosuppression is presented in the current report. The tumor was composed of large B-cell-like cells, and was positive for cluster of differentiation 138 and melanoma ubiquitous mutated-1, with ~80% of the tumor cells positive for Ki-67. The features of the tumor were as follows: Extraoral location, HIV-negative, immunoglobulin M λ-type M protein expression, light chain restriction (monoclonal) and Epstein-Barr virus-encoded small RNA-negative, which are considered to be unusual for PBL. These unusual features complicate the differentiation of PBL from other plasma cell diseases. To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient. To date, the standard treatment of PBL remains elusive, however, the most commonly administered chemotherapy treatments are CHOP [intravenous cyclophosphamide (750 mg/m(2), day 1), intravenous doxorubicin (50 mg/m(2), day 1), intravenous vincristine (1.4 mg/m(2), day 1) and prednisone (100 mg, days 1-50)]-like regimens. The patient was administered two cycles of CHOP chemotherapy for 56 days, however, ultimately succumbed as a result of disease progression. Therefore, PBL represents a diagnostic and therapeutic challenge. PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients. Furthermore, CHOP does not appear to be an optimal treatment regimen and more intensive regimens are required.

No MeSH data available.


Related in: MedlinePlus