Cytomegalovirus-Associated Hemophagocytic Syndrome

PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 280-283
Author(s):  
ELIZABETH H. DANISH ◽  
BEVERLY B. DAHMS ◽  
MARY L. KUMAR

Virus-associated hemophagocytic syndrome, first described by Risdall and co-workers in 1979,1 is a rare histiocytic proliferative syndrome characterzed by fever, hepatosplenomegaly, pancytopenia, and erythrophagocytosis by histiocytes that appear benign by histologic criteria. The clinical course and pathologic findings may be identical with another histiocytic disorder, familial erythrophagocytic lymphohistiocytosis, which occurs predominantly in infants. Diagnosis of virus-associated hemophagocytic syndrome depends entirely on evidence of concurrent viral infection, usually of the herpes group. Epstein-Barr virus has been associated with this syndrome in the few cases reported in children without underlying disease, whereas cytomegalovirus (CMV) has been implicated in immunosuppressed patients. We report a case of fatal CMV-associated hemophagocytic syndrome which occurred in a previously healthy infant.

2003 ◽  
Vol 2 (2) ◽  
pp. 86-89
Author(s):  
A. P. Pomogaeva ◽  
O. I. Galaktionova

The aim of this investigation is to assess variants of Epstein—Barr viral infection course in infectious mononucleosis nidus. There have been examined 5 groups of preschool institutions, in each there was found 1 case of typical infectious mononucleosis caused by Epstein—Barr virus. Total number of children under observation was 67: 5 children with typical (clinical) infectious mononucleosis and 62 children being in contact under the disease. Analyses of life history, clinical examination, peripheral blood examination, serologic marker spectrum of EBV- and HIV-infections and factors of immune system cellular component have been made during investigation. It was found that 1 child in a contact group was absolutely healthy, 53 children were virus carriers and 8 children had atypical infectious mononucleosis. Upon the investigation results the formation of infectious mononucleosis nidus has been proved and their peculiarities have been found.


2020 ◽  
Author(s):  
Jinjin Shi ◽  
Chu Chu ◽  
Min Yu ◽  
Dandan Zhang ◽  
Yuqin Li ◽  
...  

Abstract Objectives This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). Methods The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively. Results Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively. Conclusion The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.


Blood ◽  
1997 ◽  
Vol 90 (3) ◽  
pp. 1186-1191 ◽  
Author(s):  
Marcelo G. Horenstein ◽  
Roland G. Nador ◽  
Amy Chadburn ◽  
Elizabeth M. Hyjek ◽  
Giorgio Inghirami ◽  
...  

Primary effusion (body cavity–based) lymphoma (PEL) is a recently recognized subtype of malignant lymphoma that exhibits distinctive clinical and biological features, most notably its usual infection with the Kaposi's sarcoma–associated herpesvirus (KSHV). The vast majority of cases also contain Epstein-Barr virus (EBV). This dual viral infection is the first example of a consistent dual herpesviral infection in a human neoplasm and provides a unique model to study viral interactions. We analyzed the pattern of EBV latent gene expression to determine the pathogenic role of this agent in PELs. We examined five PELs coinfected with EBV and KSHV by reverse transcription-polymerase chain reaction (RT-PCR), in situ hybridization, and immunohistochemistry. EBER1 mRNA, a consistent marker of viral latency, was positive in all PEL cases, although at lower levels than in the non-PEL controls due to EBER1 expression by only a variable subset of lymphoma cells. Qp-initiated mRNA, encoding only EBNA1 and characteristic of latencies I and II, was positive in all PEL cases. Wp- and Cp-initiated mRNAs, encoding all EBNAs and characteristic of latency III, were negative in all cases. LMP1 mRNA, expressed in latencies II and III, was present in three cases of PEL, although at very low levels that were not detectable at the protein level by immunohistochemistry. Low levels of LMP2A mRNA were detected in all cases. BZLF1, an early-intermediate lytic phase marker, was weakly positive in four cases, suggesting a productive viral infection in a very small proportion of cells, which was confirmed by ZEBRA antigen expression. Therefore, PELs exhibit a restricted latency pattern, with expression of EBNA1 in all cases, and low LMP1 and LMP2A levels.


2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Bo Zhang ◽  
Xia Wang ◽  
Xiaoyan Tian ◽  
Yongping Cai ◽  
Xingwang Wu

Aim. To improve the identification and computed tomography (CT) diagnostic accuracy of chronic active Epstein-Barr virus (EBV)-associated enteritis (CAEAE) by evaluating its CT findings and clinical manifestation. Methods. The data of three patients with pathologically and clinically confirmed CAEAE who underwent CT enterography (CTE) were retrospectively reviewed from January 2018 to October 2019. The following data were evaluated: imaging characteristics (length of involvement, pattern of mural thickening, pattern of attenuation, perienteric abnormalities), clinical symptoms, endoscopic records, laboratory examinations, and pathologic findings. Results. Based on CT findings, two patients demonstrated segmental bowel wall thickening (involvement length >6 cm), asymmetric thickening, layered attenuation, fat stranding, and adenopathy, whereas the remaining one had no positive finding. The endoscopic results of all patients showed numerous irregular ulcers in the colon, and one patient had a focal esophageal ulcer. The major clinical symptoms were abdominal pain (n=3), retrosternal pain (n=1), fever (n=3), diarrhea (n=2), hematochezia (n=1), and adenopathy (n=3). The main laboratory examination indicators were increased serum EBV DNA load (n=1) and increased inflammatory markers (n=3). With regard to the main pathologic findings, all patients showed positive EBV-encoded RNA (EBER) situ hybridization in the colonic biopsy specimen, with one patient being positive in the esophagus. Conclusion. CAEAE is rare and is usually misdiagnosed as inflammatory bowel disease (IBD). The imaging features of CAEAE overlap with those of Crohn’s disease and ulcerative colitis. The presence of segmental and asymmetric bowel wall thickening, layered attenuation, and fat stranding in the CTE image may be helpful in differentiating CAEAE from IBD.


Head & Neck ◽  
2004 ◽  
Vol 26 (9) ◽  
pp. 815-822 ◽  
Author(s):  
Antti A. Mäkitie ◽  
Patricia P. Reis ◽  
Jonathan Irish ◽  
Tong Zhang ◽  
Soo F. Chin ◽  
...  

Author(s):  
Kenzo Koizumi ◽  
Yuiko Tsukada ◽  
Daisuke Ito ◽  
Suketaka Momoshima ◽  
Shinichiro Okamoto ◽  
...  

Blood ◽  
1972 ◽  
Vol 40 (5) ◽  
pp. 754-758 ◽  
Author(s):  
Hans W. von Heyden ◽  
George E. Moore

Abstract Lymphoblasts appearing in immunosuppressed patients after bone marrow transfusion are compared to those that can be established in vitro as permanent lymphoid cell lines. It is suggested that Epstein-Barr virus (EBV) could be responsible for the recurrent "lymphoblastic leukemia" in these patients and that the transplanted cells may be a clone of nonmalignant cells that has become capable of growing without normal restraints. It is important that in future patients the transplanted cells be characterized as to morphology, chromosome constitution, relative clonability and transplantability, the presence of EBV, T or B cell-like traits, and their growth potential in immunosuppressed heterologous hosts. The antibody titer to EBV should be measured before and after leukocyte transfusion.


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