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B-Cell-Rich T-Cell Lymphoma Associated with Epstein-Barr Virus-Reactivation and T-Cell Suppression Following Antithymocyte Globulin Therapy in a Patient with Severe Aplastic Anemia.

Hanaoka N, Murata S, Hosoi H, Shimokado A, Mushino T, Kuriyama K, Hatanaka K, Nishikawa A, Kurimoto M, Sonoki T, Muragaki Y, Nakakuma H - Hematol Rep (2015)

Bottom Line: We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia.The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma.This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology/Oncology.

ABSTRACT
B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV)-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

No MeSH data available.


Related in: MedlinePlus

Severe aplastic anemia with aggressive LPD and EBV-reactivation. (A) Bone marrow biopsy showing markedly hypocellular marrow. (B) Immunosuppressive therapy, LPD, and EBV-proliferation. The T-cell population of lymphocytes disappeared after ATG therapy. PSL, prednisolone; CsA, ciclosporin; ATG, antithymocyte globulin; FCN, foscarnet; IVIG, intravenous injection of immunoglobulins; LDH, lactate dehydrogenase; sIL-2R, soluble IL-2 receptor.
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fig001: Severe aplastic anemia with aggressive LPD and EBV-reactivation. (A) Bone marrow biopsy showing markedly hypocellular marrow. (B) Immunosuppressive therapy, LPD, and EBV-proliferation. The T-cell population of lymphocytes disappeared after ATG therapy. PSL, prednisolone; CsA, ciclosporin; ATG, antithymocyte globulin; FCN, foscarnet; IVIG, intravenous injection of immunoglobulins; LDH, lactate dehydrogenase; sIL-2R, soluble IL-2 receptor.

Mentions: A 69-year-old woman with acquired AA was admitted for ATG-based immunosuppressive therapy. On admission, she did not exhibit any signs of lymphadenopathy or hepatomegaly. Laboratory tests are shown in Table 1. She had undergone total gastrectomy, partial pancreas head resection, and splenectomy for gastric cancer four years previously, and had been diagnosed with idiopathic AA based on findings such as hypoplastic fatty marrow (Figure 1A) with a normal karyotype in the absence of an abnormal infiltrate including lymphoid neoplasms, myelodysplasia, infections, or rheumatic diseases six months prior to her admission. She was treated initially with cicrosporin (CsA) and metenolone acetate, and subsequently with rabbit ATG, CsA, and prednisolone. The T-cell population of lymphocytes disappeared after the therapy (Figure 1B). She developed fever, hypoglycemia, and marked lymphocytosis with the reactivation of EBV and cytomegalovirus on day 54 after this treatment (Figure 1B). EBV-reactivation was confirmed by an increase in serum EBV-DNA (300×103 copies/mL) and the results of serological tests (positive for both IgG to the EBV capsid antigen and antibody to the EBV nuclear antigen). She also exhibited rapidly fatal complications associated with hepatomegaly, renal dysfunction, and an infection with Stenotrophomonas maltophilia prior to initiating treatment with rituximab (Table 1). Clinically, she was diagnosed with aggressive LPD and EBV-reactivation.


B-Cell-Rich T-Cell Lymphoma Associated with Epstein-Barr Virus-Reactivation and T-Cell Suppression Following Antithymocyte Globulin Therapy in a Patient with Severe Aplastic Anemia.

Hanaoka N, Murata S, Hosoi H, Shimokado A, Mushino T, Kuriyama K, Hatanaka K, Nishikawa A, Kurimoto M, Sonoki T, Muragaki Y, Nakakuma H - Hematol Rep (2015)

Severe aplastic anemia with aggressive LPD and EBV-reactivation. (A) Bone marrow biopsy showing markedly hypocellular marrow. (B) Immunosuppressive therapy, LPD, and EBV-proliferation. The T-cell population of lymphocytes disappeared after ATG therapy. PSL, prednisolone; CsA, ciclosporin; ATG, antithymocyte globulin; FCN, foscarnet; IVIG, intravenous injection of immunoglobulins; LDH, lactate dehydrogenase; sIL-2R, soluble IL-2 receptor.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4591498&req=5

fig001: Severe aplastic anemia with aggressive LPD and EBV-reactivation. (A) Bone marrow biopsy showing markedly hypocellular marrow. (B) Immunosuppressive therapy, LPD, and EBV-proliferation. The T-cell population of lymphocytes disappeared after ATG therapy. PSL, prednisolone; CsA, ciclosporin; ATG, antithymocyte globulin; FCN, foscarnet; IVIG, intravenous injection of immunoglobulins; LDH, lactate dehydrogenase; sIL-2R, soluble IL-2 receptor.
Mentions: A 69-year-old woman with acquired AA was admitted for ATG-based immunosuppressive therapy. On admission, she did not exhibit any signs of lymphadenopathy or hepatomegaly. Laboratory tests are shown in Table 1. She had undergone total gastrectomy, partial pancreas head resection, and splenectomy for gastric cancer four years previously, and had been diagnosed with idiopathic AA based on findings such as hypoplastic fatty marrow (Figure 1A) with a normal karyotype in the absence of an abnormal infiltrate including lymphoid neoplasms, myelodysplasia, infections, or rheumatic diseases six months prior to her admission. She was treated initially with cicrosporin (CsA) and metenolone acetate, and subsequently with rabbit ATG, CsA, and prednisolone. The T-cell population of lymphocytes disappeared after the therapy (Figure 1B). She developed fever, hypoglycemia, and marked lymphocytosis with the reactivation of EBV and cytomegalovirus on day 54 after this treatment (Figure 1B). EBV-reactivation was confirmed by an increase in serum EBV-DNA (300×103 copies/mL) and the results of serological tests (positive for both IgG to the EBV capsid antigen and antibody to the EBV nuclear antigen). She also exhibited rapidly fatal complications associated with hepatomegaly, renal dysfunction, and an infection with Stenotrophomonas maltophilia prior to initiating treatment with rituximab (Table 1). Clinically, she was diagnosed with aggressive LPD and EBV-reactivation.

Bottom Line: We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia.The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma.This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology/Oncology.

ABSTRACT
B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV)-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

No MeSH data available.


Related in: MedlinePlus