title: Occurrence of Epstein-Barr virus-associated plasmacytic lymphoproliferative disorder after antithymocyte globulin therapy for aplastic anemia: a case report with review of the literature.
abstract: It is well established that patients with immunosuppression have a higher risk of development of lymphoproliferative disorders (LPDs), and Epstein-Barr virus (EBV) is associated with development of LPDs. Aplastic anemia (AA) is an immune-mediated hematological disorder, and immunosuppression therapy (IST), such as antithymocyte globulin (ATG), is widely used for treatment of AA. However, occurrence of LPD without bone marrow transplantation has been extremely rarely documented in patients with IST for AA. Herein, we report the 6th documented case of EBV-associated LPD after IST for AA and review the clinicopathological features of this extremely rare complication. A 46-year-old Japanese female was admitted for evaluation of progressive pancytopenia. Bone marrow biopsy revealed fatty marrow with marked decrease of trilineage cells, and bone marrow aspiration demonstrated no dysplastic changes. IST with rabbit ATG was administered, after which, she developed high fever. Bone marrow aspiration showed increase of atypical plasma cells with mildly enlarged nuclei and irregular nuclear contour. These atypical plasma cells were EBER-positive. Accordingly, a diagnosis of EBV-positive plasmacytic LPD was made. Most cases of LPDs are B-cell origin, and plasmacytic LPD is a rare subtype. The current report is the second case of plasmacytic LPD in patients with IST for AA. Therefore, detailed histopathological and immunohistochemical analyses are needed for correct diagnosis and treatment, and additional studies are needed to clarify the clinicopathological features of EBV-LPD after IST for AA.
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ryota
authors.initials: R
authors.lastname: Nakanishi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hodohara
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Okuno
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Miyuki
authors.initials: M
authors.lastname: Yoshii
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Horinouchi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ayaka
authors.initials: A
authors.lastname: Shirakawa
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ayumi
authors.initials: A
authors.lastname: Harada
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Muneo
authors.initials: M
authors.lastname: Iwai
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Yoshida
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Kagotani
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshida
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Okabe
mesh_terms: D000741:Anemia, Aplastic / Q000188:drug therapy*; D000961:Antilymphocyte Serum / Q000009:adverse effects* / Q000627:therapeutic use*; D000998:Antiviral Agents / Q000627:therapeutic use; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology*; D008875:Middle Aged; D016896:Treatment Outcome; D028761:Withholding Treatment
chemical_list: D000961:Antilymphocyte Serum; D000998:Antiviral Agents
keywords: Epstein-Barr virus-associated lymphoproliferative disorder; antithymocyte globulin therapy; aplastic anemia