The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients.
Chronic CD8(+) T-cell expansions can result in parotid gland swelling and other organ infiltration in HIV-infected patients, or in persistent cytopenias. We report 14 patients with a CD8+ T-cell expansion to better characterize the clinical spectrum of this ill-defined entity. Patients (9 women/5 me...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2014-01-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC3950180?pdf=render |
| _version_ | 1828205108440072192 |
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| author | Etienne Ghrenassia Louise Roulin Aude Aline-Fardin Christophe Marzac Frédéric Féger Julie Gay Jérome Pacanowski Alexandre Hertig Paul Coppo |
| author_facet | Etienne Ghrenassia Louise Roulin Aude Aline-Fardin Christophe Marzac Frédéric Féger Julie Gay Jérome Pacanowski Alexandre Hertig Paul Coppo |
| author_sort | Etienne Ghrenassia |
| collection | DOAJ |
| description | Chronic CD8(+) T-cell expansions can result in parotid gland swelling and other organ infiltration in HIV-infected patients, or in persistent cytopenias. We report 14 patients with a CD8+ T-cell expansion to better characterize the clinical spectrum of this ill-defined entity. Patients (9 women/5 men) were 65 year-old (range, 25-74). Six patients had ≥ 1 symptomatic organ infiltration, and 9 had ≥ 1 cytopenia with a CD8(+) (>50% of total lymphocyte count) and/or a CD8(+)/CD57(+) (>30% of total lymphocyte count) T-cell expansion for at least 3 months. One patient had both manifestations. A STAT3 mutation, consistent with the diagnosis of large granular lymphocyte leukemia, was found in 2 patients with cytopenia. Organ infiltration involved lymph nodes, the liver, the colon, the kidneys, the skin and the central nervous system. Three patients had a HIV infection for 8 years (range, 0.5-20 years). Two non-HIV patients with hypogammaglobulinemia had been treated with a B-cell depleting monoclonal antibody (rituximab) for a lymphoma. One patient had a myelodysplastic syndrome with colon infiltration and agranulocytosis. The outcome was favorable with efficient antiretroviral therapy and steroids in HIV-infected patients and intravenous immunoglobulins in 2/3 non-HIV patients. Six patients had an agranulocytosis of favorable outcome with granulocyte-colony stimulating factor only (3 cases), cyclophosphamide, methotrexate and cyclosporine A, or no treatment (1 case each). Three patients had a pure red cell aplasia, of favorable outcome in 2 cases with methotrexate and cyclosporine A; one patient was unresponsive. Chronic CD8(+) T-cell expansions with organ infiltration in immunocompromised patients may involve other organs than parotid glands; they are non clonal and of favorable outcome after correction of the immune deficiency and/or steroids. In patients with bone marrow infiltration and unexplained cytopenia, CD8(+) T-cell expansions can be clonal or not; their identification suggests that cytopenias are immune-mediated. Our results extend the clinical spectrum of chronic CD8(+) T-cell expansions. |
| first_indexed | 2024-04-12T12:34:26Z |
| format | Article |
| id | doaj.art-9f0d99df0e9747a2bbbd5ef81947b1f3 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-04-12T12:34:26Z |
| publishDate | 2014-01-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-9f0d99df0e9747a2bbbd5ef81947b1f32022-12-22T03:32:56ZengPublic Library of Science (PLoS)PLoS ONE1932-62032014-01-0193e9150510.1371/journal.pone.0091505The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients.Etienne GhrenassiaLouise RoulinAude Aline-FardinChristophe MarzacFrédéric FégerJulie GayJérome PacanowskiAlexandre HertigPaul CoppoChronic CD8(+) T-cell expansions can result in parotid gland swelling and other organ infiltration in HIV-infected patients, or in persistent cytopenias. We report 14 patients with a CD8+ T-cell expansion to better characterize the clinical spectrum of this ill-defined entity. Patients (9 women/5 men) were 65 year-old (range, 25-74). Six patients had ≥ 1 symptomatic organ infiltration, and 9 had ≥ 1 cytopenia with a CD8(+) (>50% of total lymphocyte count) and/or a CD8(+)/CD57(+) (>30% of total lymphocyte count) T-cell expansion for at least 3 months. One patient had both manifestations. A STAT3 mutation, consistent with the diagnosis of large granular lymphocyte leukemia, was found in 2 patients with cytopenia. Organ infiltration involved lymph nodes, the liver, the colon, the kidneys, the skin and the central nervous system. Three patients had a HIV infection for 8 years (range, 0.5-20 years). Two non-HIV patients with hypogammaglobulinemia had been treated with a B-cell depleting monoclonal antibody (rituximab) for a lymphoma. One patient had a myelodysplastic syndrome with colon infiltration and agranulocytosis. The outcome was favorable with efficient antiretroviral therapy and steroids in HIV-infected patients and intravenous immunoglobulins in 2/3 non-HIV patients. Six patients had an agranulocytosis of favorable outcome with granulocyte-colony stimulating factor only (3 cases), cyclophosphamide, methotrexate and cyclosporine A, or no treatment (1 case each). Three patients had a pure red cell aplasia, of favorable outcome in 2 cases with methotrexate and cyclosporine A; one patient was unresponsive. Chronic CD8(+) T-cell expansions with organ infiltration in immunocompromised patients may involve other organs than parotid glands; they are non clonal and of favorable outcome after correction of the immune deficiency and/or steroids. In patients with bone marrow infiltration and unexplained cytopenia, CD8(+) T-cell expansions can be clonal or not; their identification suggests that cytopenias are immune-mediated. Our results extend the clinical spectrum of chronic CD8(+) T-cell expansions.http://europepmc.org/articles/PMC3950180?pdf=render |
| spellingShingle | Etienne Ghrenassia Louise Roulin Aude Aline-Fardin Christophe Marzac Frédéric Féger Julie Gay Jérome Pacanowski Alexandre Hertig Paul Coppo The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. PLoS ONE |
| title | The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. |
| title_full | The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. |
| title_fullStr | The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. |
| title_full_unstemmed | The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. |
| title_short | The spectrum of chronic CD8+ T-cell expansions: clinical features in 14 patients. |
| title_sort | spectrum of chronic cd8 t cell expansions clinical features in 14 patients |
| url | http://europepmc.org/articles/PMC3950180?pdf=render |
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