This syndrome is classified as familial or acquired. We report a case of hps associated with cytomegalovirus cmv infection in a patient with rheumatoid arthritis ra. Cytomegalovirus infectionassociated hemophagocytic syndrome. Two patients with the acquired immunodeficiency syndrome and disseminated cytomegalovirus infection developed hypercalcemia associated with suppressed parathyroid gland activity. We report two cases of cytomegalovirus cmv viraemia resulting in severe pneumonitis and associated haemophagocytic syndrome manifesting in patients with inflammatory bowel disease, on stable doses of azathioprine in clinical remission.
There is no standard therapy for vahs and the clinical course is variable. Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of. Nov 30, 2015 hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a rare and life. Hiv clinical presentation in the acute stage is variable and some of its virological and immunological aspects are not completely understood. Subsequently, hlh has been reported in association with a variety of infections, and the term reactive hemophagocytic syndrome. However, the reactive and hereditary forms of the disease are difficult to distinguish. Hlh has since been associated with a variety of viral, bacterial, fungal, and parasitic infections, as well as collagenvascular diseases 711 and malignancies, particularly tcell lymphomas 1214.
In spite of prompt diagnosis and treatment, the infant expired due to. There is enough evidence that supports the gravity of the cmv infection. A 29yearold man with hiv infection was referred to our department because of a 1month history of lowgrade fever and fatigue. Infectionassociated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever. Hemophagocytic lymphohistiocytosis hlh is a serious, aggressive, and lifethreatening syndrome caused by a dysfunction of the immune system and is characterized by excessive immune system activation. 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.
We utilized a recently developed murine model of cytomegalovirus associated. Drug reaction with eosinophilia and systemic symptoms. Virusinfected t lymphocytes undergo clonal proliferation. The presumed mechanism for his multi organ inflammatory syndrome is the altered immune response induced by the overwhelming bacterial and viral infection he had had, mycoplasma pneumonia and cmv in our case. Of great importance in our patient, treatment of cmv infection.
Infection associated hemophagocytic syndrome infectionassociated hemophagocytic syndrome iahs. Infections associated with haemophagocytic syndrome the. Introduction hemophagocytic syndrome, also known as hemophagocytic. Nephrotic syndrome associated with hemophagocytic syndrome. Primary hlh tends to be of genetic etiology, while secondary hlh results from other insults such as. Hemophagocytic lymphohistiocytosis in a patient with advanced hiv and cytomegalovirus infection amir anabtawi, md, reem alkilany, md, and mary e. Nephrotic syndrome associated with hemophagocytic syndrome o thaunat1, m delahousse2, f fakhouri1, f martinez1. We are presenting this case aiming to raise the awareness for this rare combination.
Hemophagocytic syndrome hps is clinically defined as a combination of fever, liver dysfunction, coagulation abnormalities, pancytopenia, progressive macrophage proliferation throughout the reticuloendothelial system, and cytokine overproduction, and may be primary or secondary to infectious, autoimmune, and tumoral diseases. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis hlh is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells. Infections associated with hemophagocytic syndrome. The smears were also examined for lymphocytes, abnormal cells, histiocytes, hemophagocytosis and parasites. A rare case of cytomegalovirus infection induced hemophagacytic. Hemophagocytic syndrome associated with cytomegalovirus. Cmvassociated hlh has been reported in a variety of immunocompromised states including solid organ transplantation, infection, and autoimmune disease. Virus associated hemophagocytic syndrome is a nonneoplastic, generalized histiocytic proliferation with prominent hemophagocytosis associated with a systemic viral infection. Here, we report a case of epsteinbarr virus ebv associated hps after scrub ty phus infection. We present a case of hemophagocytic syndrome secondary to.
Autoimmuneassociated hemophagocytic syndromemacrophage. Pregnancyrelated hemophagocytic lymphohistiocytosis. A case of hemophagocytic syndrome in a patient with fulminant. Infectious workup revealed cytomegalovirus viremia, and the patient met hlh criteria with impressive hyperferritinemia of 15 432 ngml. Introduction hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a rare and lifethreatening disease that is frequently underrecognized. Infection associated hemophagocytic syndrome virus associated hemophagocytic syndrome herpesvirus hiv other viruses adenovirus mumps hepatitis a, b, c enterovirus parvovirus b19 human tlymphotropic virus influenza flavivirus dengue fever measles h1n1 other infections associated with hemophagocytic syndrome bacterial parasitic fungal. Hemophagocytic lymphohistiocytosis in a patient with. Hlh may be primary genetic etiology or secondary to malignancies, autoimmune disease, or infections most commonly viral. Hemophagocytic syndrome hps is an uncommon hematological disorder that manifests as fever, splenomegaly, and jaundice, with hemophagocytosis in the bone marrow and other tissues pathologically.
Most cases of hiv associated reactive hemophagocytic syndrome have been reported in patients with advanced stages of hiv and to our knowledge, there are only 8 cases in the english literature presenting during acute hiv infection. The syndrome is characterized by fever, altered mental status, hepatosplenomegaly, cytopenia, liver dysfunction. Bone marrow aspiration and biopsy showed findings consistent with haemophagocytic syndrome hps, and immunohistochemical assessment showed cytomegalovirus cmv infection. Neither patient had evidence of a malignant, endocrinologic, granulomatous, or drugrelated cause for hypercalcemia. The most consistent association is with viral infections. The diagnosis includes a spectrum of inherited or acquired defects in cytotoxic lymphocyte function, often with uncontrolled infections. Hence, cmv associated haemophagocytic syndrome in context of azathioprine therapy for ulcerative colitis was established. Hemophagocytic lymphohistiocytosis hlh is characterized by severe immune activation and deregulation resulting in extreme and often lifethreatening inflammation 1. The study found cmv infection in 14% of the pooled patients. Disseminated cytomegalovirus associated hemophagocytic. Haemophagocytic syndrome has been associated with a variety of viral, bacterial, fungal, and parasitic infections.
Hemophagocytic lymphohistiocytosis hlh is a rare and often deadly syndrome characterized by severe inflammation and cytokine dysregulation. Having both cmv and mycoplasma pneumonia in one patient diagnosed with hlh is a rare event. Hemophagocytic lymphohistiocytosis hlh is a disease that results from severe. Chandima divithotawela, the prince charles hospital, rode road, chermside, queensland 4032, australia. It is characterized by proliferation and activation of benign histiocytes. Mouse cytomegalovirus infection in balbc mice resembles virus associated secondary hemophagocytic. The prevalence of cytomegalovirus cmv infection in ulcerative colitis uc patients is approximately 16%. Pdf hemophagocytic lymphohistiocytosis hlh is a rare and often deadly syndrome characterized by severe inflammation and cytokine. Secondary hps is associated with malignancy and infection, especially viral infection. Lung involvement in hlh has received little attention.
Hemophagocytic lymphohistiocytosis associated with. Chandima divithotawela, the prince charles hospital. Infection associated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever, hepatosplenomegaly, and cytopeniamost commonly, thrombocytopenia and anemia. Hence, cmv associated hemophagocytic syndrome in the context of recent corticotherapy for crohns disease was established. Per guidelines from the histiocyte society hlh94, therapy traditionally involves remission treatment.
We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome aids and burkitts lymphoma. It results in histiocytic proliferation with significant hemophagocytic activity. In most cases, treatment of the underlying condition promotes complete. Hlh is a systemic inflammatory syndrome associated with impaired cytotoxic. Secondary hlh is associated with infections, hematologic malignancy, autoimmune disease, and drugs. A case of hemophagocytic syndrome in a patient with. After 2 weeks of antiviral therapy, she became afebrile, with rapid. The patient was diagnosed with virus associated hemophagocytic syndrome and was successfully treated with antiviral therapy consisting of intravenous ganciclovir, gamma globulin, and granulocytecolony stimulating factor. This diversity has prompted the suggestion that hlh secondary to an underlying medical illness should be designated reactive hemophagocytic syndrome. Infection associated hemophagocytic histiocytosis and its effect on hematological parameters marrow in general, cellularity and maturation of erythropoiesis, myelopoiesis and megakaryocytes. Acquired hlh due to viral infections also known as virus associated hemophagocytic syndromeor vahs is most commonly associated with ebstein barr virus ebv, cytomegalovirus cmv. Citeseerx document details isaac councill, lee giles, pradeep teregowda.
It is most common in children but can affect patients of any age. This case illustrates successful treatment of hlh associated with cytomegalovirus pneumonitis with the interleukin 1 inhibitor anakinra. This case highlights the complexity of pregnancyrelated hlh and identifies some key areas in the management of this disease that should be addressed in order to improve survival in this potentially fatal disease. Hemophagocytic syndrome hps is a clinicopathologic entity characterized by systemic proliferation of benign hemophagocytic histiocytes in the bone marrow, associated with fever, cytopenias, liver dysfunction and coagulopathy. The underlying hemophagocytosis occurs primarily in the bone marrow, liver and lymph nodes. Familial and acquired hemophagocytic lymphohistiocytosis. We report a case of acute cmv infection complicated by cmv ileitis and hemophagocytic syndrome. We report the successful management of this syndrome with ganciclovir, corticosteroids and the new interleukin 1 inhibitor anakinra. Hemophagocytic lymphohistiocytosis hlh is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation. This article describes lung involvement in hlh and assesses whether it affects the prognosis. Disseminated cytomegalovirus associated hemophagocytic lymphohistiocytosis in an elderly patient to the editor.
Viral infections associated with the syndrome include ebv, cytomegalovirus, human herpesvirus 8 hhv8, hiv, influenza virus, parvovirus, hepatitis virus, and enterovirus. Hemophagocytic lymphohistiocytosis hlh is a disorder characterized by uncontrolled mature histiocyte proliferation. Hypercalcemia and disseminated cytomegalovirus infection in. Acquired hemophagocytic lymphohistiocytosis hlh is a lifethreatening event that usually occurs as a complication of immunodeficiency. In 1979, hlh was described in a cohort of patients who had serologic evidence of recent viral infections, and virus associated hemophagocytic syndrome was proposed as a distinct clinical entity. The exact role of the viral infection in the development of the hemophagocytic syndrome remains to be further elucidated. We report a case of hps associated with cytomegalovirus cmv infection. Cytomegalovirusassociated hemophagocytic lymphohistiocytosis. Hemophagocytic syndrome is a clinical condition characterized by the infiltration. Virus associated hemophagocytic syndrome genetic and rare. Hiv itself may be a trigger for hps, but the clinical course associated with cytomegalovirus cmv.
A 22yearold man with a history of crohns disease presented with fever, nausea, and vomiting. It is characterized by proliferation and activation of benign histiocytes, causing dysfunction of various organs. Infection associated hemophagocytic syndrome medicnote. Steps taken to alleviate under reporting of transfusion reactions at a public sector.
Hiv associated hps can occur at any stages of hiv disease and requires diverse differential. Hemophagocytic syndromes and infection volume 6, number. Acute cytomegalovirus infection related to the immunosuppressive therapy, resulting in hemophagocytosis syndrome and neutropenic fever was diagnosed. We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome. 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. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal syndrome that results from inappropriate activation of the immune system. Pulmonary involvement in patients with hemophagocytic. The patients characteristics are described in table 1.
Morphologic demonstration of hemophagocytosis is 1 of 8 criteria used to diagnose hlh, but it may not always be apparent at disease onset. Pdf acute cytomegalovirus cmv infection associated with. Hemophagocytic lymphohistiocytosis in a patient with advanced. Pdf pregnancyrelated hemophagocytic lymphohistiocytosis. A rare complication of cmv infection in crohns disease. May 08, 20 virus associated hemophagocytic syndrome is a very serious complication of a viral infection.
Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors in bone marrow and other tissues. Mouse cytomegalovirus infection in balbc mice resembles virus associated secondary hemophagocytic lymphohistiocytosis and shows a pathogenesis distinct from primary hemophagocytic lymphohistiocytosis ellen brisse, maya imbrechts, karen put, anneleen avau, tania mitera. Infection associated hemophagocytic histiocytosis and its. They were monitored for cmv reactivation once to twice a week. Epsteinbarr virus associated hemophagocytic syndrome. Most people dont know they have cmv because it rarely causes problems in healthy people if youre pregnant or if your immune system is weakened, cmv is cause for concern. Mouse cytomegalovirus infection in balbc mice resembles.
Recovery was achieved by the administration of parenteral ganciclovir, broad spectrum antibiotic and complex intensive care. Many viral agents are known to trigger hlh but cytomegalovirus cmv associated hlh is rarely described. Hereditary and acquired hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis hlh is a rare and often. A diagnosis of hemophagocytic lymphohistiocytosis hlh accompanied by cmv infection was rendered. Cureus acute cytomegalovirus cmv infection associated. Mar 01, 2006 s ir recent reports of serious cytomegalovirus cmv infections in patients receiving treatment with infliximab have appeared 1, 2. Virus associated hemophagocytic syndrome is a rare condition, precipitated by viral infection. Haemophagocytic syndrome can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. It is described as primary hlh familial hlh and secondary hlh acquired following malignancy, rheumatologic disorders, primary immune deficiencies or infection.
The mr imaging appearance of a case of virus associated hemophagocytic syndrome complicated by diffuse cns infiltration is presented. Viral infections associated with the syndrome include ebv, cytomegalovirus. Cytomegalovirus infection monitored by quantitative real. We report a case of cytomegalovirus cmv associated hemophagocytic syndrome. Cytomegalovirus associated hemophagocytic syndrome in a. The patient was started on treatment for cytomegalovirus infection that led to resolution of hlh. Multiple microbiological agents, including cytomegalovirus. Apr 10, 2014 a diagnosis of hemophagocytic lymphohistiocytosis hlh accompanied by cmv infection was rendered. There have been multiple previous reports of cytomegalovirus cmv associated hlh developing in patients with ulcerative colitis receiving azathioprine. Hemophagocytic lymphohistiocytosis hlh is a rare hyperinflammatory syndrome characterized by fever, pancytopenia and splenomegaly. We report the successful management of this syndrome. Pdf phaemophagocytic syndrome caused by cmv infection in a one month old infant is rare. We report our experience in managing a previously healthy pregnant patient who presented in the third trimester of pregnancy with hlh. Evidence for cytomegalovirusinduced haemophagocytic syndrome.
Pdf hemophagocytic syndrome secondary to cytomegalovirus. We report a case of epsteinbarr virus associated hemophagocytic syndrome. The acquired forms of hemophagocytic syndrome include infection associated hemophagocytic syndrome, historically known as histiocytic medullary reticulosis or virus associated hemophagocytic syndrome. Cytomegalovirus infection associated hemophagocytic syndrome. S ir recent reports of serious cytomegalovirus cmv infections in patients receiving treatment with infliximab have appeared 1, 2. Hemophagocytic syndromes and infection volume 6, number 6. Detection of cmv dna in blood samples was performed by quantitative rtpcr on whole blood. Debate around infectiondependent hemophagocytic syndrome. Evidence for cytomegalovirusinduced haemophagocytic. The syndrome is associated with collagen vascular diseases, malignancies and infections most frequently caused by viruses, prominently linked with epsteinbarr viral infection. There have been a small number of cases of scrub typhus associated hemophagocytic syndrome hps, most of which were treated successfully using adequate antibiotics. Hlh associated with an identifiable infectious or noninfectious etiology from its hereditary forms.
It resembles the systemic inflammatory response syndrome, and progresses to multiorgan dysfunction. Cytomegalovirus ileitis and hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis hlh is a rare yet potentially fatal clinicopathological entity because of uncontrolled immune system activation. Successful treatment of cytomegalovirus associated. Once infected, your body retains the virus for life. Infections associated with haemophagocytic syndrome are most frequently caused by viruses, particularly epsteinbarr virus ebv. We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome and burkitts lymphoma. Hemophagocytic syndrome therapy should be instituted promptly to prevent irreversible tissue damage. Patient 2 presented a cmv associated hemophagocytic syndrome. Hemophagocytic syndrome secondary to cytomegalovirus. The syndrome is characterized by fever, altered mental status, hepatosplenomegaly, cytopenia, liver dysfunction, and. Disseminated cmv associated with hlh is uncommon in adults, this case is a type of. Hemophagocytic lymphohistiocytosis hlh is not one condition but descriptive of a lifethreatening, hyperinflammatory syndrome with multiorgan involvement with a variety of triggers, both genetic and environmental.
The disease is defined by the hlh2004 criteria, requiring five of eight findings, and is further differentiated into either primary or secondary causes. Hemophagocytic syndrome an overview sciencedirect topics. Cytomegalovirusassociated hemophagocytic syndrome in a. Successful treatment of cytomegalovirus associated hemophagocytic lymphohistiocytosis with the interleukin 1 inhibitor anakinra. Hemophagocytic syndrome associated with cytomegalovirus viral infection. Mar 20, 2014 hemophagocytic lymphohistiocytosis hlh is a potentially fatal syndrome that results from inappropriate activation of the immune system. Hemophagocytic syndrome associated with cytomegalovirus infection in a severely. Acute hiv infection presenting as hemophagocytic syndrome. Hemophagocytic syndrome associatedcmv infection is an infrequent but severe condition related to. Virus associated hemophagocytic syndrome vahs is a rare complication in early cytomegalovirus cmv infection. To the best of our knowledge, finding both organisms in one case is rarely reported. Acute cytomegalovirus infectionassociated hemophagocytic. Immunosuppressive therapy is the basis of the therapy in combination with the speci. May 19, 2004 viral studies serum antibody titer and antigenemia of cytomegalovirus revealed systemic cytomegalovirus infection.
Infectionassociated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever, hepatosplenomegaly, and cytopeniamost commonly, thrombocytopenia and anemia. Autoimmune associated hemophagocytic syndromemacrophage activation syndrome 83 the reported incidence of juvenile idiopathic arthritis jia varies from 1 to 22 cases per 100,000 children, with a. Epstein barr virus associated hemophagocytic syndrome. Hiv can cause diverse opportunistic infections and secondary haemophagocytic syndrome hps. Infections associated with hemophagocytic syndrome request pdf.
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