scholarly journals Linfohistiocitose Hemofagocítica Secundária a Infeção por Epstein-Barr: Raridade e Gravidade num Adulto Imunocompetente

2019 ◽  
Vol 32 (1) ◽  
pp. 78
Author(s):  
Andreia Sofia Costa ◽  
Anusca Paixão ◽  
Henrique Santos ◽  
Fernando Salvador
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Clinical Case ◽  
Specific Treatment ◽  
Supportive Therapy ◽  
Intravascular Coagulation ◽  
Barr Virus ◽  
Life Threatening ◽  
Epstein Barr

Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.

scholarly journals Nivolumab treatment of relapsed/refractory Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis in adults

Blood ◽  
2020 ◽  
Vol 135 (11) ◽  
pp. 826-833 ◽  
Author(s):  
Pengpeng Liu ◽  
Xiangyu Pan ◽  
Chong Chen ◽  
Ting Niu ◽  
Xiao Shuai ◽  
...  
Keyword(s):  
T Cells ◽  
Cd8 T Cells ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Sequencing Analysis ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Life Threatening ◽  
Programmed Death Protein 1 ◽  
Epstein Barr

Abstract Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome triggered by EBV infection. It often becomes relapsed or refractory (r/r), given that etoposide-based regimens cannot effectively clear the virus. r/r EBV-HLH is invariably lethal in adults without allogeneic hematopoietic stem cell transplantation. Here, we performed a retrospective analysis of 7 r/r EBV-HLH patients who were treated with nivolumab on a compassionate-use basis at West China Hospital. All 7 patients tolerated the treatment and 6 responded to it. Five of them achieved and remained in clinical complete remission with a median follow-up of 16 months (range, 11.4-18.9 months). Importantly, both plasma and cellular EBV-DNAs were completely eradicated in 4 patients. Single-cell RNA-sequencing analysis showed that HLH syndrome was associated with hyperactive monocytes/macrophages and ineffective CD8 T cells with a defective activation program. Nivolumab treatment expanded programmed death protein-1–positive T cells and restored the expression of HLH-associated degranulation and costimulatory genes in CD8 T cells. Our data suggest that nivolumab, as a monotherapy, provides a potential cure for r/r EBV-HLH, most likely by restoring a defective anti-EBV response.

Coinfection with Parvovirus B19 and Epstein Barr Virus in An Adolescent with Sickle Cell Disease Manifesting As Septic Shock

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4854-4854
Author(s):  
Jyoti Panicker ◽  
Robyn Gartrell Harris ◽  
Stephen Dale Smith
Keyword(s):  
Septic Shock ◽  
Epstein Barr Virus ◽  
Parvovirus B19 ◽  
Supportive Therapy ◽  
Red Cell ◽  
Barr Virus ◽  
Ebv Reactivation ◽  
Life Threatening ◽  
Epstein Barr ◽  
Dna Pcr

Abstract Abstract 4854 Patients with Sickle Cell Disease (SCD) are at an increased risk of serious and life-threatening infections. Epstein Barr Virus (EBV) and Human Parvovirus B19 (HPV-B19) are common viral infections that are usually asymptomatic or self-limiting in healthy children. However, SCD patients can have significant morbidity and mortality caused by these infections. In patients with SCD, EBV can cause splenic rupture, thrombocytopenia, agranulocytosis, hemolytic anemia and hemophagocytic lymphocytosis while HPV-B19 can cause severe aplastic crisis, stroke, nephrotic syndrome, myocardial infarction and splenic sequestration. Coinfection with EBV and HPV-B19 has not been reported in SCD patients and there are no reports of EBV or HPV B19 causing septic shock. A 15 year old male with SCD presented with a one day history of fever, oliguria, and hypotension caused by simultaneous viremia with EBV and HPV-B19. He had been discharged 2 days previously after an admission for a vaso-occlusive crisis. He did not have pharyngitis, rash hepatosplenomegaly (spleen likely autoinfarcted) or lymphadenopathy. Peripheral smear did not show lymphocytosis, atypical lymphocytes or features of microangiopathic hemolytic anemia. Fluid resuscitation with normal saline and then with red cell transfusion was done and he was transferred to pediatric intensive care unit. He received supportive therapy for septic shock with fluids, antibiotics and red cell transfusions. Blood and urine bacterial cultures were negative and blood quantitative DNA PCR was positive for EBV (3300 copies/ml) and HPV-B19 (2.3×107 copies/ml). His EBV titers were positive for viral capsid antigen (VCA)-IgM and IgG and Epstein Barr nuclear antigen (EBNA)-IgG. He had EBV titers done 2 years previously which were positive for VCA-IgG and EBNA-IgG documenting previous primary EBV infection. The patient gradually improved with supportive therapy and was discharged on Day 8. The patient returned to Pediatric Hematology clinic on Day 19 and had resolution of signs and symptoms. Table 1 shows an overview of his blood counts and transfusion requirements.Table 1 Coinfection with EBV and HPV-B19 in healthy adults has rarely been reported. Severe aplastic anemia caused by EBV and HPV-B19 infections was reported in a previously healthy 22-year-old male. He was treated with immunosuppressive therapy followed by allogeneic bone marrow transplant (Kaptan K et al, Am J Hematol 2001). Acute HPV-B19 infection and concomitant EBV reactivation causing moderate hemophagocytic syndrome was reported in a previously healthy 37-year-old-male (Larroche C et al, Eur J Clin Microbiol Infect Dis 2002). HPV-B19 infection with EBV reactivation has not been reported in children. Sepsis from viremia is not common with either EBV or HPV-B19, however together this co-infection can be life-threatening as seen in our patient. HPV-B19 infection has been reported to produce false positive EBV VCA-IgM serology assay. Our patient had a true coinfection because he had viremia documented by EBV DNA PCR. This is the first report of viremia and septic shock associated with an acute HPV-B19 infection and EBV reactivation in a patient with SCD. WBC- White Blood Cell Count, ANC- Absolute Neutrophil Count, ALC- Absolute Lymphocyte Count, Plt- Platelet Count, Hgb- Hemoglobin, HCT- Hematocrit, Retic: Corrected Reticulocyte Count, U- Units, PRBC- Packed red blood cells, ER- Emergency room Disclosures: No relevant conflicts of interest to declare.

scholarly journals Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Neda Hashemi-Sadraei ◽  
Pimprapa Vejpongsa ◽  
Muhamed Baljevic ◽  
Lei Chen ◽  
Modupe Idowu
Keyword(s):  
Septic Shock ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Care Setting ◽  
Clinical Syndrome ◽  
Barr Virus ◽  
Life Threatening ◽  
Triggering Conditions ◽  
Epstein Barr ◽  
Delayed Recognition

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic.

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