Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review
We describe a rare case of adult T-cell leukemia characterized by an expansion of CD4+ CD8+ double-positive lymphocytes associated with human T-lymphotropic virus type 1 (HTLV-1) and a complex karyotype in a 43-year-old Caribbean male who was initially admitted to our hospital with significant letha...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Karger Publishers
2010-12-01
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| Series: | Case Reports in Oncology |
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| Online Access: | http://www.karger.com/Article/FullText/323163 |
| _version_ | 1818471779873587200 |
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| author | Shahzad Raza Seema Naik Venkat P. Kancharla Fekade Tafera Madhumati R. Kalavar |
| author_facet | Shahzad Raza Seema Naik Venkat P. Kancharla Fekade Tafera Madhumati R. Kalavar |
| author_sort | Shahzad Raza |
| collection | DOAJ |
| description | We describe a rare case of adult T-cell leukemia characterized by an expansion of CD4+ CD8+ double-positive lymphocytes associated with human T-lymphotropic virus type 1 (HTLV-1) and a complex karyotype in a 43-year-old Caribbean male who was initially admitted to our hospital with significant lethargy, visual disturbances, dysphagia, right facial palsy and numbness in both feet for 3 days. He was found to have severe hypercalcemia (15.6 mg/dl). Peripheral blood smear showed multilobulated clover-shaped nuclei. Bone marrow and CSF flow cytometries revealed abnormal monoclonal expansion of T cells positive for CD4, CD5, CD8 and CD25 but negative for CD7, CD20, CD56, CD68 and terminal deoxynucleotidyl transferase. The polymerase chain reaction analysis showed a distinct band of the T-cell receptor γ gene, revealing T-cell clonal integration of the proviral DNA of HTLV-1, thus confirming the diagnosis of acute adult T-cell leukemia/lymphoma. Cytogenetic study revealed a male karyotype with monosomy 12, unbalanced translocation 5q and 13q and additional material on 5q, 7q, 14q and 17q. The patient underwent prednisone (EPOCH) chemotherapy followed by autologous transplantation with BEAM regimen. Although patients with a rare mixed CD4+ CD8+ immunophenotype usually present with an aggressive clinical course and have a poor prognosis, our patient was able to survive for 2.5 years. |
| first_indexed | 2024-04-14T03:56:11Z |
| format | Article |
| id | doaj.art-45f416c1bc4f4c61acc1373931f228cc |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-04-14T03:56:11Z |
| publishDate | 2010-12-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-45f416c1bc4f4c61acc1373931f228cc2022-12-22T02:13:45ZengKarger PublishersCase Reports in Oncology1662-65752010-12-013348949410.1159/000323163323163Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature ReviewShahzad RazaSeema NaikVenkat P. KancharlaFekade TaferaMadhumati R. KalavarWe describe a rare case of adult T-cell leukemia characterized by an expansion of CD4+ CD8+ double-positive lymphocytes associated with human T-lymphotropic virus type 1 (HTLV-1) and a complex karyotype in a 43-year-old Caribbean male who was initially admitted to our hospital with significant lethargy, visual disturbances, dysphagia, right facial palsy and numbness in both feet for 3 days. He was found to have severe hypercalcemia (15.6 mg/dl). Peripheral blood smear showed multilobulated clover-shaped nuclei. Bone marrow and CSF flow cytometries revealed abnormal monoclonal expansion of T cells positive for CD4, CD5, CD8 and CD25 but negative for CD7, CD20, CD56, CD68 and terminal deoxynucleotidyl transferase. The polymerase chain reaction analysis showed a distinct band of the T-cell receptor γ gene, revealing T-cell clonal integration of the proviral DNA of HTLV-1, thus confirming the diagnosis of acute adult T-cell leukemia/lymphoma. Cytogenetic study revealed a male karyotype with monosomy 12, unbalanced translocation 5q and 13q and additional material on 5q, 7q, 14q and 17q. The patient underwent prednisone (EPOCH) chemotherapy followed by autologous transplantation with BEAM regimen. Although patients with a rare mixed CD4+ CD8+ immunophenotype usually present with an aggressive clinical course and have a poor prognosis, our patient was able to survive for 2.5 years.http://www.karger.com/Article/FullText/323163Acute T-cell leukemiaCyclophosphamideDoxorubicinEtoposideHuman T-lymphotropic virus type 1 (HTLV-1)PrednisoneVincristine |
| spellingShingle | Shahzad Raza Seema Naik Venkat P. Kancharla Fekade Tafera Madhumati R. Kalavar Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review Case Reports in Oncology Acute T-cell leukemia Cyclophosphamide Doxorubicin Etoposide Human T-lymphotropic virus type 1 (HTLV-1) Prednisone Vincristine |
| title | Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review |
| title_full | Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review |
| title_fullStr | Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review |
| title_full_unstemmed | Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review |
| title_short | Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review |
| title_sort | dual positive cd4 cd8 acute adult t cell leukemia lymphoma associated with complex karyotype and refractory hypercalcemia case report and literature review |
| topic | Acute T-cell leukemia Cyclophosphamide Doxorubicin Etoposide Human T-lymphotropic virus type 1 (HTLV-1) Prednisone Vincristine |
| url | http://www.karger.com/Article/FullText/323163 |
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