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