The patient was started on treatment for cytomegalovirus infection that led to resolution of hlh. Disseminated cytomegalovirus associated hemophagocytic. Immunosuppressive therapy is the basis of the therapy in combination with the speci. Successful treatment of cytomegalovirus associated hemophagocytic lymphohistiocytosis with the interleukin 1 inhibitor anakinra.
A case of hemophagocytic syndrome in a patient with. We present a case of hemophagocytic syndrome secondary to. Hemophagocytic lymphohistiocytosis hlh is a rare hyperinflammatory syndrome characterized by fever, pancytopenia and splenomegaly. In most cases, treatment of the underlying condition promotes complete.
There is no standard therapy for vahs and the clinical course is variable. Infection associated hemophagocytic histiocytosis and its. Autoimmuneassociated hemophagocytic syndromemacrophage. Hypercalcemia and disseminated cytomegalovirus infection in. The study found cmv infection in 14% of the pooled patients.
Infection associated hemophagocytic syndrome medicnote. Acute cytomegalovirus infection related to the immunosuppressive therapy, resulting in hemophagocytosis syndrome and neutropenic fever was diagnosed. 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. 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 lymphohistiocytosis associated with. Cytomegalovirus infection monitored by quantitative real. Virus associated hemophagocytic syndrome is a nonneoplastic, generalized histiocytic proliferation with prominent hemophagocytosis associated with a systemic viral infection. 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. Virusinfected t lymphocytes undergo clonal proliferation. Pdf hemophagocytic syndrome secondary to cytomegalovirus. A rare complication of cmv infection in crohns disease.
We utilized a recently developed murine model of cytomegalovirus associated. Chandima divithotawela, the prince charles hospital. We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome aids and burkitts lymphoma. Hemophagocytic lymphohistiocytosis hlh is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation. Hereditary and acquired hemophagocytic lymphohistiocytosis. The acquired forms of hemophagocytic syndrome include infection associated hemophagocytic syndrome, historically known as histiocytic medullary reticulosis or virus associated hemophagocytic syndrome. Hence, cmv associated hemophagocytic syndrome in the context of recent corticotherapy for crohns disease was established. Hemophagocytic lymphohistiocytosis hlh is a rare and often deadly syndrome characterized by severe inflammation and cytokine dysregulation. Bone marrow aspiration and biopsy showed findings consistent with haemophagocytic syndrome hps, and immunohistochemical assessment showed cytomegalovirus cmv infection. 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.
Here, we report a case of epsteinbarr virus ebv associated hps after scrub ty phus infection. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis hlh is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells. 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. Steps taken to alleviate under reporting of transfusion reactions at a public sector. Haemophagocytic syndrome can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. Pregnancyrelated hemophagocytic lymphohistiocytosis. Cytomegalovirusassociated hemophagocytic lymphohistiocytosis. This diversity has prompted the suggestion that hlh secondary to an underlying medical illness should be designated reactive hemophagocytic syndrome. We report the successful management of this syndrome. Hemophagocytic syndrome associated with cytomegalovirus infection in a severely. Virus associated hemophagocytic syndrome genetic and rare. Lung involvement in hlh has received little attention. Hemophagocytic lymphohistiocytosis hlh is a rare and often.
Secondary hps is associated with malignancy and infection, especially viral infection. There is enough evidence that supports the gravity of the cmv infection. We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome. We report a case of cytomegalovirus cmv associated hemophagocytic syndrome. There have been multiple previous reports of cytomegalovirus cmv associated hlh developing in patients with ulcerative colitis receiving azathioprine. Haemophagocytic syndrome has been associated with a variety of viral, bacterial, fungal, and parasitic infections. Apr 10, 2014 a diagnosis of hemophagocytic lymphohistiocytosis hlh accompanied by cmv infection was rendered. Evidence for cytomegalovirusinduced haemophagocytic. Evidence for cytomegalovirusinduced haemophagocytic syndrome.
Hemophagocytic syndrome is a clinical condition characterized by the infiltration. Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of. Patient 2 presented a cmv associated hemophagocytic syndrome. A 29yearold man with hiv infection was referred to our department because of a 1month history of lowgrade fever and fatigue. Pdf phaemophagocytic syndrome caused by cmv infection in a one month old infant is rare. We report a case of hps associated with cytomegalovirus cmv infection in a patient with rheumatoid arthritis ra. Debate around infectiondependent hemophagocytic syndrome. They were monitored for cmv reactivation once to twice a week. Hemophagocytic lymphohistiocytosis hlh is a disorder characterized by uncontrolled mature histiocyte proliferation. Infections associated with hemophagocytic syndrome request pdf. Chandima divithotawela, the prince charles hospital, rode road, chermside, queensland 4032, australia.
Acquired hemophagocytic lymphohistiocytosis hlh is a lifethreatening event that usually occurs as a complication of immunodeficiency. Infections associated with haemophagocytic syndrome the. Introduction hemophagocytic syndrome, also known as hemophagocytic. Successful treatment of cytomegalovirus associated.
Cytomegalovirus infection associated hemophagocytic syndrome. Drug reaction with eosinophilia and systemic symptoms. A case of hemophagocytic syndrome in a patient with fulminant. Infection associated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever, hepatosplenomegaly, and cytopeniamost commonly, thrombocytopenia and anemia. Hemophagocytic lymphohistiocytosis hlh is a disease that results from severe. 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. 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. Disseminated cytomegalovirus associated hemophagocytic lymphohistiocytosis in an elderly patient to the editor. Associated with reactivation of epsteinbarr virus andor cytomegalovirus leading to hemophagocytic syndrome in one of two patients jianhua liang1,2, hui qu1,3, xiaowen wang1,3, aiping wang1,3.
Nov 30, 2015 hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a rare and life. Having both cmv and mycoplasma pneumonia in one patient diagnosed with hlh is a rare event. There have been a small number of cases of scrub typhus associated hemophagocytic syndrome hps, most of which were treated successfully using adequate antibiotics. We report our experience in managing a previously healthy pregnant patient who presented in the third trimester of pregnancy with hlh. The syndrome is characterized by fever, altered mental status, hepatosplenomegaly, cytopenia, liver dysfunction, and. The syndrome is characterized by fever, altered mental status, hepatosplenomegaly, cytopenia, liver dysfunction. A rare case of cytomegalovirus infection induced hemophagacytic. Hlh associated with an identifiable infectious or noninfectious etiology from its hereditary forms. 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.
Hiv itself may be a trigger for hps, but the clinical course associated with cytomegalovirus cmv. This syndrome is classified as familial or acquired. Many viral agents are known to trigger hlh but cytomegalovirus cmv associated hlh is rarely described. Hemophagocytic syndromes and infection volume 6, number.
Disseminated cmv associated with hlh is uncommon in adults, this case is a type of. It results in histiocytic proliferation with significant hemophagocytic activity. It is characterized by proliferation and activation of benign histiocytes, causing dysfunction of various organs. To the best of our knowledge, finding both organisms in one case is rarely reported. Hiv can cause diverse opportunistic infections and secondary haemophagocytic syndrome hps. In spite of prompt diagnosis and treatment, the infant expired due to. It is characterized by proliferation and activation of benign histiocytes. Pdf acute cytomegalovirus cmv infection associated with. Infectious workup revealed cytomegalovirus viremia, and the patient met hlh criteria with impressive hyperferritinemia of 15 432 ngml. Hemophagocytic lymphohistiocytosis in a patient with advanced hiv and cytomegalovirus infection amir anabtawi, md, reem alkilany, md, and mary e. Hemophagocytic syndrome therapy should be instituted promptly to prevent irreversible tissue damage. Hlh is a systemic inflammatory syndrome associated with impaired cytotoxic.
Viral infections associated with the syndrome include ebv, cytomegalovirus. Infection associated hemophagocytic syndrome infectionassociated hemophagocytic syndrome iahs. We report a case of epsteinbarr virus associated hemophagocytic syndrome. Hemophagocytic syndrome an overview sciencedirect topics. 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. Hemophagocytic syndrome associatedcmv infection is an infrequent but severe condition related to.
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. Hemophagocytic lymphohistiocytosis in a patient with advanced. 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. This article describes lung involvement in hlh and assesses whether it affects the prognosis. Epsteinbarr virus associated hemophagocytic syndrome. 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. Hemophagocytic syndrome associated with cytomegalovirus viral infection. The underlying hemophagocytosis occurs primarily in the bone marrow, liver and lymph nodes. Pdf hemophagocytic lymphohistiocytosis hlh is a rare and often deadly syndrome characterized by severe inflammation and cytokine. 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. The most consistent association is with viral infections. Citeseerx document details isaac councill, lee giles, pradeep teregowda.
Cytomegalovirus ileitis and hemophagocytic syndrome. Pdf pregnancyrelated hemophagocytic lymphohistiocytosis. Neither patient had evidence of a malignant, endocrinologic, granulomatous, or drugrelated cause for hypercalcemia. Two patients with the acquired immunodeficiency syndrome and disseminated cytomegalovirus infection developed hypercalcemia associated with suppressed parathyroid gland activity. The smears were also examined for lymphocytes, abnormal cells, histiocytes, hemophagocytosis and parasites. Hemophagocytic lymphohistiocytosis hlh is a rare yet potentially fatal clinicopathological entity because of uncontrolled immune system activation. 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. We report the successful management of this syndrome with ganciclovir, corticosteroids and the new interleukin 1 inhibitor anakinra. S ir recent reports of serious cytomegalovirus cmv infections in patients receiving treatment with infliximab have appeared 1, 2. Mouse cytomegalovirus infection in balbc mice resembles virus associated secondary hemophagocytic. Mouse cytomegalovirus infection in balbc mice resembles. Subsequently, hlh has been reported in association with a variety of infections, and the term reactive hemophagocytic syndrome.
Cytomegalovirus associated hemophagocytic syndrome in a. Infections associated with hemophagocytic syndrome. 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. We are presenting this case aiming to raise the awareness for this rare combination.
Infectionassociated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever. Cytomegalovirus infectionassociated hemophagocytic syndrome. Hlh may be primary genetic etiology or secondary to malignancies, autoimmune disease, or infections most commonly viral. The patients characteristics are described in table 1. 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. Acute cytomegalovirus infectionassociated hemophagocytic. The mr imaging appearance of a case of virus associated hemophagocytic syndrome complicated by diffuse cns infiltration is presented. Hemophagocytic lymphohistiocytosis hlh is characterized by severe immune activation and deregulation resulting in extreme and often lifethreatening inflammation 1. Primary hlh tends to be of genetic etiology, while secondary hlh results from other insults such as. Familial and acquired hemophagocytic lymphohistiocytosis. Viral infections associated with the syndrome include ebv, cytomegalovirus, human herpesvirus 8 hhv8, hiv, influenza virus, parvovirus, hepatitis virus, and enterovirus.
Nephrotic syndrome associated with hemophagocytic syndrome. This case illustrates successful treatment of hlh associated with cytomegalovirus pneumonitis with the interleukin 1 inhibitor anakinra. Once infected, your body retains the virus for life. May 19, 2004 viral studies serum antibody titer and antigenemia of cytomegalovirus revealed systemic cytomegalovirus infection. The syndrome is associated with collagen vascular diseases, malignancies and infections most frequently caused by viruses, prominently linked with epsteinbarr viral infection. Cytomegalovirusassociated hemophagocytic syndrome in a. 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 rare condition, precipitated by viral infection.
We report a case of acute cmv infection complicated by cmv ileitis and hemophagocytic syndrome. A diagnosis of hemophagocytic lymphohistiocytosis hlh accompanied by cmv infection was rendered. Of great importance in our patient, treatment of cmv infection. Mar 01, 2006 s ir recent reports of serious cytomegalovirus cmv infections in patients receiving treatment with infliximab have appeared 1, 2. Introduction hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a rare and lifethreatening disease that is frequently underrecognized. Infection associated hemophagocytic histiocytosis and its effect on hematological parameters marrow in general, cellularity and maturation of erythropoiesis, myelopoiesis and megakaryocytes.
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. Hiv clinical presentation in the acute stage is variable and some of its virological and immunological aspects are not completely understood. Pulmonary involvement in patients with hemophagocytic. Multiple microbiological agents, including cytomegalovirus. Hemophagocytic syndrome secondary to cytomegalovirus. It is described as primary hlh familial hlh and secondary hlh acquired following malignancy, rheumatologic disorders, primary immune deficiencies or infection. Hlh is a systemic inflammatory syndrome associated with impaired cytotoxic activity.
Detection of cmv dna in blood samples was performed by quantitative rtpcr on whole blood. We report a case of hps associated with cytomegalovirus cmv infection. Acute hiv infection presenting as hemophagocytic syndrome. Hiv associated hps can occur at any stages of hiv disease and requires diverse differential. A 22yearold man with a history of crohns disease presented with fever, nausea, and vomiting. Per guidelines from the histiocyte society hlh94, therapy traditionally involves remission treatment.
Morphologic demonstration of hemophagocytosis is 1 of 8 criteria used to diagnose hlh, but it may not always be apparent at disease onset. Secondary hlh is associated with infections, hematologic malignancy, autoimmune disease, and drugs. We report a case of hlh associated with cytomegalovirus infection in a patient with acquiredimmunodeficiency syndrome and burkitts lymphoma. 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.
Recovery was achieved by the administration of parenteral ganciclovir, broad spectrum antibiotic and complex intensive care. It is most common in children but can affect patients of any age. Hence, cmv associated haemophagocytic syndrome in context of azathioprine therapy for ulcerative colitis was established. The prevalence of cytomegalovirus cmv infection in ulcerative colitis uc patients is approximately 16%. After 2 weeks of antiviral therapy, she became afebrile, with rapid. Epstein barr 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. Virus associated hemophagocytic syndrome vahs is a rare complication in early cytomegalovirus cmv infection. Infectionassociated hemophagocytic syndrome is a lifethreatening condition characterized by prolonged fever, hepatosplenomegaly, and cytopeniamost commonly, thrombocytopenia and anemia. Infections associated with haemophagocytic syndrome are most frequently caused by viruses, particularly epsteinbarr virus ebv.
Nephrotic syndrome associated with hemophagocytic syndrome o thaunat1, m delahousse2, f fakhouri1, f martinez1. Hemophagocytic lymphohistiocytosis in a patient with. The disease is defined by the hlh2004 criteria, requiring five of eight findings, and is further differentiated into either primary or secondary causes. 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 syndrome associated cmv infection is an infrequent but severe condition related to. It resembles the systemic inflammatory response syndrome, and progresses to multiorgan dysfunction. May 08, 20 virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Hemophagocytic syndrome associated with cytomegalovirus. The exact role of the viral infection in the development of the hemophagocytic syndrome remains to be further elucidated. Pathophysiology and epidemiology of hemophagocytic. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal syndrome that results from inappropriate activation of the immune system.