Epstein-Barr virus central nervous system involvement in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. 2022

Qingxia Yin, and Jingshi Wang, and Zhao Wang
Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing, China.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a common subtype of secondary HLH. EBV plays an important part in the course. EBV can cause central nervous system (CNS) infections, and there are few clinical studies on EBV-CNS infection in EBV-HLH patients. All patients who were diagnosed as EBV-HLH and underwent cerebrospinal fluid testing admitted to our center from January 2018 to December 2019 were retrospectively analyzed. Summarized the clinical data, evaluated treatment efficacy after intrathecal injection, and investigated the correlation between EBV-CNS infection with prognosis in EBV-HLH patients. Of 37 of 57 (64.9%) EBV-HLH patients has EBV-CNS infection. The survival of EBV-HLH patients without EBV-CNS infection was significantly better than that in EBV-CNS infection patients (P = 0.018). There were no statistically significant differences in sCD25, ferritin, ALT, AST, LDH, TB, WBC, Hb, and PLT counts between two groups (all P-values > 0.05). Higher EBV-DNA load in peripheral blood was correlated with EBV-CNS infection (P < 0.001). EBV-CNS infection is an independent risk factor affecting the survival of patients (P = 0.004). The CSF cell load of patients with and without EBV-CNS infection groups was significantly different (P = 0.024). Intrathecal injection with methotrexate combined with dexamethasone can effectively decrease CSF EBV-DNA load (P = 0.017) and CSF cell load (P = 0.025). EBV-CNS infection is an independent risk factor affecting prognosis in EBV-HLH patients. Therefore, EBV-CNS infection should cause concern for EBV-HLH patients. Cerebrospinal fluid testing is necessary for all patients. Methotrexate combined with dexamethasone intrathecal injection can be an effective treatment for EBV-CNS infection.

UI MeSH Term Description Entries
D008727 Methotrexate An antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of TETRAHYDROFOLATE DEHYDROGENASE and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA. Amethopterin,Methotrexate Hydrate,Methotrexate Sodium,Methotrexate, (D)-Isomer,Methotrexate, (DL)-Isomer,Methotrexate, Dicesium Salt,Methotrexate, Disodium Salt,Methotrexate, Sodium Salt,Mexate,Dicesium Salt Methotrexate,Hydrate, Methotrexate,Sodium, Methotrexate
D002490 Central Nervous System The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges. Cerebrospinal Axis,Axi, Cerebrospinal,Axis, Cerebrospinal,Central Nervous Systems,Cerebrospinal Axi,Nervous System, Central,Nervous Systems, Central,Systems, Central Nervous
D003907 Dexamethasone An anti-inflammatory 9-fluoro-glucocorticoid. Hexadecadrol,Decaject,Decaject-L.A.,Decameth,Decaspray,Dexasone,Dexpak,Hexadrol,Maxidex,Methylfluorprednisolone,Millicorten,Oradexon,Decaject L.A.
D004854 Herpesvirus 4, Human The type species of LYMPHOCRYPTOVIRUS, subfamily GAMMAHERPESVIRINAE, infecting B-cells in humans. It is thought to be the causative agent of INFECTIOUS MONONUCLEOSIS and is strongly associated with oral hairy leukoplakia (LEUKOPLAKIA, HAIRY;), BURKITT LYMPHOMA; and other malignancies. Burkitt Herpesvirus,Burkitt Lymphoma Virus,E-B Virus,EBV,Epstein-Barr Virus,Human Herpesvirus 4,Infectious Mononucleosis Virus,Burkitt's Lymphoma Virus,HHV-4,Herpesvirus 4 (gamma), Human,Burkitts Lymphoma Virus,E B Virus,E-B Viruses,Epstein Barr Virus,Herpesvirus, Burkitt,Infectious Mononucleosis Viruses,Lymphoma Virus, Burkitt,Mononucleosis Virus, Infectious,Mononucleosis Viruses, Infectious
D005293 Ferritins Iron-containing proteins that are widely distributed in animals, plants, and microorganisms. Their major function is to store IRON in a nontoxic bioavailable form. Each ferritin molecule consists of ferric iron in a hollow protein shell (APOFERRITINS) made of 24 subunits of various sequences depending on the species and tissue types. Basic Isoferritin,Ferritin,Isoferritin,Isoferritin, Basic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D051359 Lymphohistiocytosis, Hemophagocytic A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive. Familial Hemophagocytic Lymphocytosis,Hemophagocytic Lymphohistiocytosis, Familial,Hemophagocytic Syndrome, Infection-Associated,Hemophagocytic Syndrome, Reactive,Hemophagocytic Syndromes,Reactive Hemophagocytic Syndrome,Erythrophagocytic Lymphohistiocytosis, Familial,Familial Erythrophagocytic Lymphohistiocytosis,Familial Hemophagocytic Histiocytosis,Familial Hemophagocytic Lymphohistiocytosis,Familial Hemophagocytic Reticulosis,Familial Histiocytic Reticulosis,Hemophagocytic Lymphohistiocytosis Familial -1,Hemophagocytic Lymphohistiocytosis, Familial, 1,Hemophagocytic Reticulosis, Familial,Hemophagocytic Syndrome,Primary Hemophagocytic Hymphohistiocytosis,Primary Hemophagocytic Lymphohistiocytosis,Reticulosis, Familial Histiocytic,Erythrophagocytic Lymphohistiocytoses, Familial,Familial Erythrophagocytic Lymphohistiocytoses,Familial Hemophagocytic Histiocytoses,Familial Hemophagocytic Lymphocytoses,Familial Hemophagocytic Lymphohistiocytoses,Familial Hemophagocytic Reticuloses,Familial Histiocytic Reticuloses,Hemophagocytic Histiocytoses, Familial,Hemophagocytic Histiocytosis, Familial,Hemophagocytic Hymphohistiocytoses, Primary,Hemophagocytic Hymphohistiocytosis, Primary,Hemophagocytic Lymphocytoses, Familial,Hemophagocytic Lymphocytosis, Familial,Hemophagocytic Lymphohistiocytoses,Hemophagocytic Lymphohistiocytoses, Familial,Hemophagocytic Lymphohistiocytoses, Primary,Hemophagocytic Lymphohistiocytosis,Hemophagocytic Lymphohistiocytosis Familial 1,Hemophagocytic Lymphohistiocytosis, Primary,Hemophagocytic Reticuloses, Familial,Hemophagocytic Syndrome, Infection Associated,Histiocytic Reticuloses, Familial,Histiocytic Reticulosis, Familial,Histiocytoses, Familial Hemophagocytic,Histiocytosis, Familial Hemophagocytic,Hymphohistiocytoses, Primary Hemophagocytic,Hymphohistiocytosis, Primary Hemophagocytic,Infection-Associated Hemophagocytic Syndrome,Lymphocytoses, Familial Hemophagocytic,Lymphocytosis, Familial Hemophagocytic,Lymphohistiocytoses, Familial Erythrophagocytic,Lymphohistiocytoses, Familial Hemophagocytic,Lymphohistiocytoses, Hemophagocytic,Lymphohistiocytoses, Primary Hemophagocytic,Lymphohistiocytosis, Familial Erythrophagocytic,Lymphohistiocytosis, Familial Hemophagocytic,Lymphohistiocytosis, Primary Hemophagocytic,Primary Hemophagocytic Hymphohistiocytoses,Primary Hemophagocytic Lymphohistiocytoses,Reticuloses, Familial Hemophagocytic,Reticuloses, Familial Histiocytic,Reticulosis, Familial Hemophagocytic
D020031 Epstein-Barr Virus Infections Infection with human herpesvirus 4 (HERPESVIRUS 4, HUMAN); which may facilitate the development of various lymphoproliferative disorders. These include BURKITT LYMPHOMA (African type), INFECTIOUS MONONUCLEOSIS, and oral hairy leukoplakia (LEUKOPLAKIA, HAIRY). EBV Infections,Epstein-Barr Virus Infection,Herpesvirus 4 Infections, Human,Human Herpes Virus 4 Infections,Human Herpesvirus 4 Infections,Infections, EBV,Infections, Epstein-Barr Virus,EBV Infection,Epstein Barr Virus Infection,Epstein Barr Virus Infections,Infection, EBV,Infection, Epstein-Barr Virus,Virus Infection, Epstein-Barr,Virus Infections, Epstein-Barr

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