High-dose dexamethasone therapy for severe thrombocytopenia and neutropenia induced by EBV infectious mononucleosis

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Immune thrombocytopenic purpura (ITP) is a hematologic disorder characterized by autoimmune-mediated platelet destruction. Although prednisone (or prednisolone) is generally used for treatment of ITP, high-dose dexamethasone therapy is also effective and leads to rapid recovery of platelet count [1]. In contrast, optimal management of secondary ITP has not been well established. Here, we present a case of significant thrombocytopenia and neutropenia accompanied with Epstein–Barr virus (EBV)induced infectious mononucleosis (IM). A 36-year-old male visited our hospital with 2-week history of sore throat and fever. Physical examination showed cervical lymphadenopathy without hepatosplenomegaly. Peripheral blood findings were as follows: hemoglobin 13.5 g/dL, white blood cell count (WBC) 7.1 9 10/L (22.5% neutrophils, 1.5% monocytes, 6.0% lymphocytes, and 70.0% atypical lymphocytes), platelet count 71 9 10/L, aspartate aminotransferase 177 IU/L, alanine aminotransferase 240 IU/L, and lactate dehydrogenase 564 IU/L. The serological examination for EBV infection revealed the following results: the antibodies for viral capsid antigen (VCA) immunoglobulin (Ig) G was 940, VCA IgM and anti-EB virus nuclear antigen (EBNA) IgG were negative. Also, serum EBV DNA was increased to 4.0 9 10 copies/L. He was diagnosed as EBV-induced IM. His symptom disappeared in a week. However, platelet count continued to decrease, followed by decreased WBC, as shown in Fig. 1. About 60 days after the first symptom appeared, peripheral blood counts were WBC 1.4 9 10/L with 30% neutrophils and platelet count 12 9 10/L. Reticulated platelets were increased to 12.6%. The patient’s serum was positive for anti-neutrophil antibody and platelet-associated IgG (PAIgG) was increased. Antineutrophil antibody was detected by flow cytometry method. Neutrophils from normal donors were incubated with serum from the patient or pooled sera from normal volunteers as a negative control. Then, membrane-bound IgG and IgM antibodies were detected using fluorescein isothiocyanate (FITC)-labeled goat anti-human immunoglobulin. The fluorescence intensity was plotted and judged for positivity. Furthermore, bone marrow was slightly hypercellular, with increased megakaryocytes. Based on these findings, a diagnosis of autoimmune neutropenia and ITP secondary to EBV infection was done. Because thrombocytopenia prolonged and gradually decreased for about 2 months (minimum platelet count of 12 9 10/L), we started treatment with high-dose dexamethasone therapy (40 mg/day for consecutive 4 days orally) and then stopped without tapering. His platelet count increased gradually to 55 9 10/L in 2 weeks after the treatment. Also, neutrophil count improved rapidly and exceeded above 2.0 9 10/L in 3 days. Currently, 4 months after the therapy, he is well with platelet count 100 9 10/L and neutrophil count about 1.0 9 10/L. No adverse effects were noted during and after the therapy. Our patient developed typical symptoms suggestive of IM. Although negative VCA IgM antibody is inconsistent with the present infection, it is reported that VCA IgM antibody by fluorescent antibody test might be negative in 30% of patients with initial EBV infection [2]. In this case, negative EBNA IgG and positive EBV DNA in peripheral blood strongly suggest primary EBV infection. EBV-induced IM is sometimes accompanied by mild Y. Kagoya A. Hangaishi T. Takahashi Y. Imai M. Kurokawa (&) Department of Hematology and Oncology, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan e-mail: kurokawa-tky@umin.ac.jp