After completing this article, readers should be able to. Epstein barr virus associated hlh is almost universally fatal if untreated, with death usually resulting from hemorrhage, infection, or multiorgan failure 64,65. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by. Epstein barr virus ebv is now thought to be one of the major causes for the development of this unique syndrome. Pathogenic link between hydroa vacciniforme and epsteinbarr. Outline the epidemiology of primary epstein barr virus ebv infection. Despite the aggressive treatment, which included antibiotics, vasopressors, iv immunoglobulins, and. Fatal diagnosis of hemophagocytic lymphohistiocytosis in a. Chronic active epsteinbarr virus infection genetic and. Virus associated hemophagocytic syndrome genetic and. Symptomatic primary epsteinbarr virus infection is a usually selflimiting illness in adolescents. Epsteinbarr virusassociated hemophagocytic lymphohistiocyt. Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, hodgkins lymphoma, and epsteinbarr virus chelsea taylor schwartz, christopher b schmitt abstract introduction. An 18yearold girl was admitted to our hospital with a history of edema in the lower limbs and.
Pathology outlines hemophagocytic lymphohistiocytosis. Epstein barr virus ebvrelated hemophagocytic lymphohistiocytosis ebvhlh is a. Lateonset fatal epsteinbarr virusassociated hemophagocytic. As a part of a themed collection of articles on ebv infection and human primary immune deficiencies. A 9yearold white boy developed a fatal primary epsteinbarr virus ebv infection while receiving chemotherapy for acute lymphoblastic leukemia in remission. Stevensjohnson syndrome complicated by fatal hemophagocytic. Epsteinbarr virus lmp1 inhibits the expression of sap. Individuals with xlinked lymphoproliferative disease are susceptible to severe epsteinbarr virus ebv infections that are often fatal. Fatal epsteinbarr virus infection in a case of familial. Acquired hlh is most frequently associated with epstein barr virus infection but also has been associated with dengue 3.
Fatal septic shock in a patient with hemophagocytic. Ebvassociated hlh may mimic tcell lymphoma and is treated with cytotoxic chemotherapy, while hemophagocytic syndromes associated with. Haemophagocytic syndrome is a highly fatal disease if untreated. Jan 26, 2004 haemophagocytic syndrome is a serious disorder, often related to epsteinbarr virus ebv or other infectious agents. This case demonstrates that fatal ebv associated hemophagocytic syndrome hps can occur more than 1 year after cbt. The viral load in peripheral blood from patients with epsteinbarr virus ebv associated hemophagocytic syndrome was measured by realtime quantitative pcr and compared with that in infectious. Hemophagocytic lymphohistiocytosis hlh, also known as haemophagocytic lymphohistiocytosis british spelling, and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. Hemophagocytic lymphohistiocytosis, epsteinbarr virus, abatacept, rheumatoid arthritis. This report is the first of a case of lateonset ebv associated hps following cbt. Hemophagocytic syndromes and infection volume 6, number. Early etoposide treatment has been confirmed to be effective in children. Almost invariably fatal if left untreated familial hemophagocytic lymphohistiocytosis tends to recur within the first year of life in 70 80% of cases am j clin pathol 20.
Virological and immunological characteristics of a 19yearold japanese female with fatal outcome with epsteinbarr virusassociated hemophagocytic syndrome. Chronic active epsteinbarr virus infection caebv associated with hemophagocytic syndrome hps and extranodal natural killer nktcell lymphoma enkl is a rare lifethreatening disorder. Pdf cytokine profile in fatal human immunodeficiency virus. Cytokine profile in fatal human immunodeficiency virus tuberculosis epstein barr virus associated hemophagocytic syndrome. The ambiguous boundary between ebvrelated hemophagocytic. However, it is unclear whether the same treatment is useful in adults. Fatal epsteinbarr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin11 mutation.
This case report suggests that specific ebvrelated clinical and virological management should be considered when treating a patient with. Hodgkins lymphoma, epsteinbarr virus reactivation and. Epstein barr virus, hemophagocytic syndrome, fatal infectious mononucleosis introduction hemophagocytic syndromes hps may be divided into different clinical categories, consisting of 1 a sporadic disorder, 2 a. Describe and explain the physiologic basis of the clinical features of infectious mononucleosis. Ebv associated hemophagocytic lymphohistiocytosis ebvhlh, chronic. Stevensjohnson syndrome and hemophagocytic syndrome. May 10, 2019 symptomatic primary epstein barr virus infection is a usually selflimiting illness in adolescents. Ebv genomes were detected in all the patients by southern blot hybridization or the polymerase chain reaction. Jul 01, 2010 epsteinbarr virus ebv is a ubiquitous and predominantly b lymphotrophic human. We describe two unusual cases of epsteinbarr virus infection that were complicated by the virusassociated hemophagocytic syndrome, predominantly involving the spleen. Five of the six patients had serologic evidence of primary ebv infection at the onset of. Hyperproduction of cytokines, including interferon. The acquired forms of hemophagocytic syndrome include infection associated hemophagocytic syndrome, historically known as histiocytic medullary reticulosis or virus associated hemophagocytic syndrome, which is a benign, potentially reversible but often fatal disorder that occurs primarily in immunocompromised patients.
We describe six japanese children with fatal epsteinbarr virus ebvassociated hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis hlh is a rapidly fatal condition characterized by excessive immune activation. Here, we report a case of epstein barr virus ebv associated hps after scrub typhus infection that was not. We report a case of epsteinbarr virus associated hemophagocytic syndrome in a 19 year old boy who responded to treatment with steroids and etoposide. Kikuta h1, sakiyama y, matsumoto s, ohishi t, nakano t. We describe the case of a 17 year old female who developed fatal haemophagocytic syndrome hps one month following acute infection caused by epsteinbarr virus ebv. Haemophagocytic syndrome is a serious disorder, often related to epsteinbarr virus ebv or other infectious agents. Fatal hemophagocytic lymphohistiocytosis associated with. This phenomenon is an important finding in patients with hemophagocytic syndrome. We report a case of epsteinbarr virus ebv primo infection with the development of successive infectious mononucleosis, hemophagocytic lymphohistiocytosis, and bcell lymphoproliferative disorder in a patient treated with azathioprine for crohns disease.
Familial hemophagocytic lymphohistiocytosis fhl is a fatal disease of early infancy caused by defective natural killer cell activity and is characterized. Fatal epsteinbarr virus infection in a child with acute. Histopathological findings at the height of the proliferative phase of the illness were compatible with a virally induced hemophagocytic syndrome. Despite initiation of treatment and reduction of ebv load, laboratory signs of hps as severe cytopenia, hypofibrinogenemia, hyperferritinemia and hypertriglyceridemia persisted. Here, we report a case of epsteinbarr virus ebvassociated hps after scrub typhus infection that was not. Epidermal cell necrosis with direct epidermal infiltration of epsteinbarr virus ebvencoded small nuclear rnapositive t lymphocytes in a patient with ebvassociated haemophagocytic syndrome. The hemophagocytic syndrome, described in hiv positive patients, is generally associated with other viruses, especially the epstein barr virus, or to other infections 2,3,7,10. The hemophagocytic syndrome, described in hiv positive patients, is generally associated with other viruses, especially the epsteinbarr virus, or. Epsteinbarr virus ebv is a member of the herpesvirus family that can infect humans. Clinical features and treatment of natural killert cell lymphoma associated with hemophagocytic syndrome. Reactive hps has been described in association with infectious agents, but epsteinbarr virus ebv is the most commonly associated infection, and ebvassociated hps is almost universally fatal.
Epsteinbarr virus associated hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis hlh is a rare, potentially lethal disorder, cha. Reactive hps has been described in association with infectious agents, but epstein barr virus ebv is the most commonly associated infection, and ebv associated hps is almost universally fatal. Introduction hemophagocytic lymphohistiocytosis hlh is a rare and oftenfatal hyperinflammatory syndrome caused by impairment in the downregulation of immune cells. Haemophagocytic syndrome associated with pyogenic bacteria can sometimes carry a better prognosis than virus. Epstein barr virus associated hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis, also known as haemophagocytic lymphohistiocytosis british spelling, and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. However, it can be fatal for some patients because of acute renal failure, acute respiratory distress, and septic shock 3, 4. Despite initiation of treatment and reduction of ebv load, laboratory signs of hps as severe cytopenia, hypofibrinogenemia, hyperferritinemia and hypertriglyceridemia persisted, and the patient died of multiorgan failure. Hodgkins lymphoma, epsteinbarr virus reactivation and fatal. Pdf a fatal ebv related hemophagocytic lymphohistiocytosis. The clinical course is often fatal, despite specific treatment for infection and for hemophagocytic syndrome with immunosuppressive drugs.
Fatal epsteinbarr virus primo infection in a 25yearold. This disease is easily misdiagnosed because of its varied presentations. Haemophagocytic syndrome is caused by a dysregulation in natural killer tcell function, resulting in activation and proliferation of lymphocytes or histiocytes with uncontrolled haemophagocytosis and cytokine overproduction. Fatal epsteinbarr virus primo infection in a 25yearold man. Epsteinbarr virusassociated hlh is almost universally fatal if untreated, with death usually resulting from hemorrhage, infection, or multiorgan failure 64,65. Ebv infections are very common youve probably already contracted the virus without even knowing it. Chronic active epsteinbarr virus infection caebv is a very rare complication of an epstein barr virus ebv. Epsteinbarr virus ebv is a ubiquitous and predominantly b lymphotrophic human. Chronic active epsteinbarr virus infection associated. Fatal hemophagocytic lymphohistiocytosis associated with epstein. Cytokine profile in fatal human immunodeficiency virus. Hemophagocytic lymphohistiocytosis hlh cyclosporine.
Haemophagocytic syndrome or haemophagocytic lymphohistiocytosis is a rare disease that is often fatal despite treatment. Virus associated hemophagocytic syndrome genetic and rare. Hemophagocytic syndrome an overview sciencedirect topics. A nearly fatal primary epsteinbarr virus infection. Cytokine profile in fatal human immunodeficiency virus tuberculosis epsteinbarr virus associated hemophagocytic syndrome. An 11yearold girl presented with chronic, bloody diarrhea.
Ebv infection of natural killer nk and t cells is likely to be a rare event and is strongly associated with a spectrum of lymphoproliferations characterized by the pathognomic presence of monoclonal ebv in the t andor nk cells. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages. Most people about 95% by adulthood become infected by ebv at some point of their lives and never have any health problems. This case demonstrates that fatal ebvassociated hemophagocytic syndrome hps can occur more than 1 year after cbt. Mar 27, 2019 epsteinbarr virus ebv is a member of the herpesvirus family that can infect humans.
Frequently an underlying immune abnormality or a t. Hemophagocytic lymphohistiocytosis hlh is a rare and oftenfatal hyperinflammatory. This report describes a fatal case of acquired hlh that was apparently triggered by infection with denv3. We present a case of an adolescent who had been receiving azathioprine for inflammatory bowel disease for four years and developed a lifethreatening primary epstein barr virus infection successfully treated with rituximab. Mar 01, 2005 after completing this article, readers should be able to. Ebvassociated hemophagocytic lymphohistiocytosis ebv. Epsteinbarr virus associated hemophagocytic syndrome. Allogeneic bone marrow transplantation for active epstein. The epsteinbarr virus ebv can infect b cells, epithelial cells, and t or natural killer nk cells, resulting in a spectrum of benign and malignant human diseases. The viral load in peripheral blood from patients with epstein barr virus ebv associated hemophagocytic syndrome was measured by realtime quantitative pcr and compared with that in infectious.
The poor prognosis of this syndrome suggests that patients should be treated initially with combination chemotherapy and immunotherapy, regardless of whether they are thought to have. Hemophagocytic lymphohistiocytosis, epstein barr virus, abatacept, rheumatoid arthritis. Virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Epsteinbarr virus, abatacept, rheumatoid arthritis. Both patients were young adult men who presented with fever, pancytopenia, and hepatosplenomegaly. Epsteinbarr virus associated hemophagocytic lymphohistiocytosis presenting as fever of unknown origin with fatal outcome. Both had prompt symptomatic and hematologic improvement following splenectomy. Signs and symptoms of virus associated hemophagocytic syndrome, include high fever, liver problems, enlarged liver and spleen, coagulation factor abnormalities, decreased red or white blood cells and platelets pancytopenia, and a buildup of histiocytes, a type of immune cell, in various tissues in. Cytokine profile in fatal human immunodeficiency virustuberculosisepsteinbarr virusassociated hemophagocytic syndrome. The syndrome is associated with collagen vascular diseases, malignancies and infections most frequently caused by viruses, prominently linked with epsteinbarr viral infection. Acquired hlh is more frequently found in adults and is commonly secondary to infections, malignancies, or autoimmune diseases. Epsteinbarr virus, hemophagocytic syndrome, fatal infectious mononucleosis introduction hemophagocytic syndromes hps may be divided into different clinical categories, consisting of 1 a sporadic disorder, 2 a. Histologic findings of fatal epsteinbarr virusassociated subcutaneous lymphoma with hemophagocytosis a.
Hlh can occur as a familial or sporadic acquired disorder. Fatal infectious mononucleosis and virusassociated hemophagocytic syndrome. Some people with ebv will develop infectious mononucleosis or other illnesses, and will recover from their illness with no other problems. Outline the epidemiology of primary epsteinbarr virus ebv infection. Infections associated with haemophagocytic syndrome the. Fatal epsteinbarr virusassociated hemophagocytic syndrome. Epsteinbarr virus ebv is a ubiquitous virus that infects nearly all people worldwide without serious sequela. Chronic active epsteinbarr virus infection and virus. The patient developed an acute febrile illness in august 2012 during a 1month vacation in new mexico.
Pathogenic link between hydroa vacciniforme and epstein. A fatal ebv related hemophagocytic lymphohistiocytosis hlh in. The viral load in peripheral blood from patients with epsteinbarr virus ebvassociated hemophagocytic syndrome was measured by realtime quantitative pcr and compared with that in infectious. May 08, 20 virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Hps is a rare complication of scrub typhus, although it can be treated using adequate antibiotics. Epsteinbarr virus associated hemophagocytic syndrome after. The authors describe the case of a young woman who developed a clinical pictures resembling a septic shockrelated multiple organ dysfunction syndrome a couple of months after having been diagnosed suffering from a hemophagocytic lymphohistiocytosis associated with an infectious mononucleosis. However, for patients who have genetic diseases which predispose them to the development of hemophagocytic lymphohistiocytosis hlh, ebv infection is a lifethreatening problem. Epidermal cell necrosis with direct epidermal infiltration of epstein barr virus ebvencoded small nuclear rnapositive t lymphocytes in a patient with ebv associated haemophagocytic syndrome. Introduction hemophagocytic lymphohistiocytosis hlh is a rare and often fatal hyperinflammatory syndrome caused by impairment in the downregulation of immune cells. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated. Additionally, vahs is often associated with fatal infectious mononucleosis im. The acquired forms of hemophagocytic syndrome include infectionassociated hemophagocytic syndrome, historically known as histiocytic medullary reticulosis or virusassociated hemophagocytic syndrome, which is a benign, potentially reversible but often fatal disorder that occurs primarily in immunocompromised patients.
Pdf cytokine profile in fatal human immunodeficiency. Ebvassociated hemophagocytic lymphohistiocytosis ebvhlh, chronic. We describe six japanese children with fatal epsteinbarr virus ebv associated hemophagocytic syndrome. Epstein barr virus associated hemophagocytic lymphohistiocytosis is a fulminant, potentially fatal disorder, mostly occurring in patients without an underlying immune deficiency and characterized. Epsteinbarr virus ebv is now thought to be one of the major causes for the development of this unique syndrome. Hemophagocytic syndromes and infection volume 6, number 6. Five of the six patients had serologic evidence of primary ebv infection at the onset of their diseases. Hemophagocytic lymphohistiocytosis hlh is a rare hyperinflammatory syndrome with high rates of mortality. We describe the case of a 17 year old female who developed fatal haemophagocytic syndrome hps one month following acute infection caused by epstein barr virus ebv. Fatal hemophagocytic lymphohistiocytosis associated with epsteinbarr virus infection.
Epsteinbarr virusassociated tlymphoproliferative disease. Epsteinbarr virusassociated hemophagocytic syndrome and. The infection spontaneously converted to complete aplasia of the bone marrow and lymph. Although ebvassociated hlh carries a high mortality rate, quick diagnosis and appropriate treatment can subside the disease. We report a case of epstein barr virus ebv primo infection with the development of successive infectious mononucleosis, hemophagocytic lymphohistiocytosis, and bcell lymphoproliferative disorder in a patient treated with azathioprine for crohns disease. The combination of haemophagocytosis and hodgkins lymphoma seems to be rare. We present a case of an adolescent who had been receiving azathioprine for inflammatory bowel disease for four years and developed a lifethreatening primary epsteinbarr virus infection successfully treated with rituximab. This case report suggests that specific ebvrelated clinical and virological management should be considered when. Describe and explain the physiologic basis of the common complications of infectious mononucleosis. Epsteinbarr virus ebvassociated haemophagocytic syndrome. Jun 07, 2019 chronic active epstein barr virus infection caebv is a very rare complication of an epstein barr virus ebv. Epsteinbarr virusinduced hemophagocytic lymphohistiocytosis.
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