1. AbstractTo describe the clinical evolution of a case of Epstein-Barr virus infection simulating polymyositis from initial visit to final death
2. Case ReportA 17-year-old Chinesinfection from clinical findings and the antibody titer particular to EBV, EBER in the tissue and viral DNA in the blood. The patient’s clinical condition deteriorated with worsening cytopenia and liver function, decreasing fibrinogen, high serum ferritin and LDH. Patient was treated empirically with intravenous Meropenem and Caspofungin Acetate. A repeated bone marrow examination showed significant hemophagocytic activity. Hemophagocytic syndrome related gene test were negative. He finally developed fulminant hemophagocytic syndrome, rapidly deteriorated and died due to multiorgan failure in July 21st 2019.e boy came to hospital in Sep 2018 for unregularly symmetrical muscle twitches and stiffness of limbs which disappeared within half an hour without medication. He had acne-form eruptions and edema of the face with migratory headache, oral ulcers and transient low-grade fever two months ago. In January 2019, he complained of right forearm swelling. He was otherwise feeling well with no systemic symptoms. Her family history was normal. Laboratory investigations revealed bicytopenia (total white cell count 2.62 109 /L and Red Blood Cell count 3.36 1012/L), abnormal liver function tests (AST 372 U/L [15–40], ALT 138 U/L [3-35]), raised lactate dehydrogenase (LDH) 1,160 U/L [71–231], and raised muscle enzymes (creatinine kinase 14,889 U/L [24– 184] and Myoglobin (1,359.0 U/L [0–70.0]). C-reactive protein was not raised and blood culture showed no bacterial growth. A [18F] fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan revealed splenomegaly and hepatomegaly changes. There were diffuse multiple FDG-avid muscle lesions scattered in the neck, chest, waist, buttock and infection from clinical findings and the antibody titer particular to EBV, EBER in the tissue and viral DNA in the blood. The patient’s clinical condition deteriorated with worsening cytopenia and liver function, decreasing fibrinogen, high serum ferritin and LDH. Patient was treated empirically with intravenous Meropenem and Caspofungin Acetate. A repeated bone marrow examination showed significant hemophagocytic activity. Hemophagocytic syndrome related gene test were negative. He finally developed fulminant hemophagocytic syndrome, rapidly deteriorated and died due to multiorgan failure in July 21st 2019.
3. Discussion Infection with Epstein-Barr virus (EBV) is common and induces a broad spectrum of illness. Approximately 90 to 95 percent of adults are EBV seropositive worldwide; however, in a large public university in the United States, the seroprevalence of EBV antibodies among entering freshman declined from 64 percent in 2006 to 52 percent in 2012 [1]. The popularity of myositis or hemophagocytic syndrome is very rare and unknown till now. Not to say the combination of EBV associated myositis and hemophagocytic syndrome. In this case, however, despite a clinical picture of myositis, the cause of the muscle involvement was due to infiltration by EBV positive lymphocytes. EBV infection characteristically involve infectious mononucleosis. There are about 5 cases of B cell [2-7] lymphoma and 8 cases of T cell [8, 9] lymphoma reported before associated with both EBV and myositis as well as some gastric cancer, thyroid cancer and nasopharyngeal carcinoma [10-12]. Also, there are a few cases associated with muscle pathological evidence of myositis with chronic EBV infection [13, 14]. Almost all these patients went to death because of infection, DIC or multi-organ failure. What is striking in this case is that our patient had extensive and generalized muscle involvement and also hemophagocytic syndrome. Our patient also had an extremely fulminant clinical course and he died just 8 months after initial presentation of the forearm swelling. This case also highlights several clinicopathologic diagnostic challenges. Clinically, this unusual case of EBV infection mimicked polymyositis and is firstly presented with initial muscle involvement and developed into hemophagocytic syndrome. The unusual clinical course of this patient offers different, although to a great extent speculative, explanations regarding the pathogenesis the interplay between EBV, myositis and hemophagocytic syndrome. Early diagnosis is important as EBV infection with predominant muscle involvement is associated with a fulminant clinical course. Histologically, the diagnosis on the muscle biopsy was challenging for several reasons. Firstly, the lymphoid infiltrate comprised mainly small lymphocytes without significant atypia. Secondly, this patient had no immune-disorder history. Thirdly, there were no autoantibodies including anti-Jo-1 antibodies, or extramuscular symptoms suggestive of polymyositis or dermatomyositis. Hence, performing a limited IHC without EBER, will not be helpful to diagnose EBV infection involving muscle. Although a variety of therapeutic modalities have been applied, prolonged severe EBV infection is unlikely to be successfully treated. It is not known what could be the exact cause of myositis in these patients. In 1986 Walker and Jeffrey searched the protein chain sequence mimicry of EBV and muscle proteins and put forward a hypothesis that may be the mechanism of the autoimmunity that invoke the virus associated myositis [15]. It may be a direct virus induced toxicity or, on the other hand, mediated by possible myotoxic effect of the antibodies elaborated as a consequence of the EBV infection. We hope future research will throw some light on this issue.
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Li W .A Rare Case of EBV Infection Mimicking Polymyositis with Hemophagocytic Syndrome-Diagnostic Challenges and Pitfalls . Annals of Clinical and Medical Case Reports 2021