An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow
Abstract Background Indolent T-cell proliferative disorder of the GIT is a rare and provisional entity in the revised WHO 2016 classification. The patients usually have prolonged survival with persistent disease even without any treatment. Case presentation The 46 years old male patient has been fol...
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| Format: | Article |
| Language: | English |
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BMC
2018-10-01
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| Series: | Diagnostic Pathology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13000-018-0762-4 |
| _version_ | 1818899473522229248 |
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| author | Xingen Wang Chi-Sing Ng Cuimin Chen Guangyin Yu Weihua Yin |
| author_facet | Xingen Wang Chi-Sing Ng Cuimin Chen Guangyin Yu Weihua Yin |
| author_sort | Xingen Wang |
| collection | DOAJ |
| description | Abstract Background Indolent T-cell proliferative disorder of the GIT is a rare and provisional entity in the revised WHO 2016 classification. The patients usually have prolonged survival with persistent disease even without any treatment. Case presentation The 46 years old male patient has been followed up for more than 6 years without chemotherapy. Repeated gastrointestinal biopsies showed expansion of the lamina propria extending to the submucosa by small to medium sized lymphocytes with minimal cytologic atypia. The lymphoid cells were positive for CD3, CD43, TIA-1, CD2, CD7 and the B-cell marker CD20; but negative for CD4, CD8, PAX5, CD56, cyclinD1, granzyme (GraB) and Epstein Barr virus-encoded RNA (EBER). Ki-67(MIB1) index was less than 10%. Molecular tests demonstrated a clonal rearrangement for T-cell receptor γ (TCR γ) gene but immunoglobulin chain (IgH, IgK, IgL) gene remained germline. Recognition of possible aberrant CD20 expression in indolent T-cell LPD is important to avoid potential diagnostic pitfall and improper treatment. Conclusions We present an unusual case of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression, Recognition of this unusual immunophenotype of indolent T-cell LPD of GI helps to eschew misdiagnosis of B-cell and other high grade lymphomas and inappropriate aggressive treatment. |
| first_indexed | 2024-12-19T19:48:32Z |
| format | Article |
| id | doaj.art-f31539aa434a4e92b483890bb3b980a3 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-19T19:48:32Z |
| publishDate | 2018-10-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-f31539aa434a4e92b483890bb3b980a32022-12-21T20:08:05ZengBMCDiagnostic Pathology1746-15962018-10-011311710.1186/s13000-018-0762-4An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrowXingen Wang0Chi-Sing Ng1Cuimin Chen2Guangyin Yu3Weihua Yin4Department of Pathology, Shenzhen Hospital of Peking UniversityDepartment of pathology, St. Teresa’s HospitalDepartment of Pathology, Shenzhen Hospital of Peking UniversityDepartment of Pathology, Shenzhen Hospital of Peking UniversityDepartment of Pathology, Shenzhen Hospital of Peking UniversityAbstract Background Indolent T-cell proliferative disorder of the GIT is a rare and provisional entity in the revised WHO 2016 classification. The patients usually have prolonged survival with persistent disease even without any treatment. Case presentation The 46 years old male patient has been followed up for more than 6 years without chemotherapy. Repeated gastrointestinal biopsies showed expansion of the lamina propria extending to the submucosa by small to medium sized lymphocytes with minimal cytologic atypia. The lymphoid cells were positive for CD3, CD43, TIA-1, CD2, CD7 and the B-cell marker CD20; but negative for CD4, CD8, PAX5, CD56, cyclinD1, granzyme (GraB) and Epstein Barr virus-encoded RNA (EBER). Ki-67(MIB1) index was less than 10%. Molecular tests demonstrated a clonal rearrangement for T-cell receptor γ (TCR γ) gene but immunoglobulin chain (IgH, IgK, IgL) gene remained germline. Recognition of possible aberrant CD20 expression in indolent T-cell LPD is important to avoid potential diagnostic pitfall and improper treatment. Conclusions We present an unusual case of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression, Recognition of this unusual immunophenotype of indolent T-cell LPD of GI helps to eschew misdiagnosis of B-cell and other high grade lymphomas and inappropriate aggressive treatment.http://link.springer.com/article/10.1186/s13000-018-0762-4Indolent T-cell lymphoproliferative disorderGastrointestinal tractAberrant CD20 expression |
| spellingShingle | Xingen Wang Chi-Sing Ng Cuimin Chen Guangyin Yu Weihua Yin An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow Diagnostic Pathology Indolent T-cell lymphoproliferative disorder Gastrointestinal tract Aberrant CD20 expression |
| title | An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow |
| title_full | An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow |
| title_fullStr | An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow |
| title_full_unstemmed | An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow |
| title_short | An unusual case report of indolent T-cell lymphoproliferative disorder with aberrant CD20 expression involving the gastrointestinal tract and bone marrow |
| title_sort | unusual case report of indolent t cell lymphoproliferative disorder with aberrant cd20 expression involving the gastrointestinal tract and bone marrow |
| topic | Indolent T-cell lymphoproliferative disorder Gastrointestinal tract Aberrant CD20 expression |
| url | http://link.springer.com/article/10.1186/s13000-018-0762-4 |
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