Ppt hemophagocytic syndromes powerpoint presentation. Hemophagocytic syndromes and infection volume 6, number. Access to society journal content varies across our titles. Pdf hemophagocytic syndromes and infection researchgate. Ppt hemophagocytic powerpoint presentation free to. Recommendations for the management of hemophagocytic. Characterized by benign proliferation of the mature histiocyte. Hemophagocytic lymphohistiocytosis hlh, a rare but potentially fatal syndrome of immune hyperactivation, may be an underrecognized immunerelated adverse event irae. Hemophagocytic syndromes hemophagocytic lymphohistiocytosis, hlh represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Although ebv is the most common cause of infectionassociated hlh, recognition that at least some of the deaths associated with avian. 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. Hemophagocytic lymphohistiocytosis hemophagocytic syndromes hemophagocytic lymphohistiocytosis hlh falls into two categories. Hemophagocytic syndromes in adult intensive care units. Hemophagocytic lymphohistiocytosis hlh is a lifethreatening syndrome that occurs as a complication in many clinical settings.
It is sometimes difficult to establish the diagnosis of hemophagocytic lymphohistiocytosis hlh, and the combination of the physical symptoms and certain laboratory tests is required. Hemophagocytic syndromes hpss are rare, lifethreatening conditions characterized by. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperin. In general, hlh is a syndrome of pathologic immune activation with clinical manifestations of extreme inflammation. Hemophagocytic syndrome hps, also known as hemophagocytic lymphohistiocytosis hlh, is a potentially lifethreatening immune system. Hemophagocytic syndromes hpss including hemophagocytic lymphohistiocytosis hlh in adults. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a heterogenic syndrome, which leads to an acute, lifethreatening inflammatory reaction.
Patients with early relapse of primary hemophagocytic. The primary genetic form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, whereas the secondary acquired form is most frequent in adults. Hemophagocytic lymphohistiocytosis hlh has become more widely recognized in adults, with all ages affected. A set of diagnostic criteria was recommended by the histiocyte society for use in the hlh2004 research protocol, and this was revised in 2007. Discussion hemophagocytic syndromehemophagocytic lymphohistiocytosishlh it is a rare ds. Hemophagocytic lymphohistiocytosis linkedin slideshare. Hemophagocytic syndromes hemophagocytic lymphohistiocytosis, hlh represent a severe. Infectionand malignancyassociated hemophagocytic syndromes.
Familial hlh is inherited in an autosomal recessive manner. Hemophagocytic lymphohistiocytosis associated with. Hemophagocytic lymphohistiocytosis hlh is a rapidly progressive, lifethreatening syndrome of excessive immune activation. 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. Hlh appears to affect all ages, although the hereditary and sporadic cases are reported primarily in children. Depending on the etiology, hlh can be divided into genetic i. Hemophagocytic lymphohistiocytosis hlh is a lifethreatening hyperinflammatory syndrome and not an independent disease.
If you have problems viewing pdf files, download the latest version of adobe reader. The hlh disorders may be primary inheritedfamilial or secondary such as in response to specific types of infections infectionassociated hemophagocytic syndrome. Hemophagocytic lympohhistiocytosis and other hemophagocytic disorders. Familial hemophagocytic lymphohistiocytosis genetic and.
In 10 patients the diagnosis was fhlh, in 3 patients gs and in 5 patients chs. Hlh2004 diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Hemophagocytic syndromes and infection, beth israel deaconess medical center, boston, massachusetts, usa. Hlh represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. A free powerpoint ppt presentation displayed as a flash slide show on. Hemophagocytic lymphohistiocytosis hlh is a hyperinflammatory syndrome with high mortality even with appropriate treatment. Hemophagocytic lymphohistiocytosis hlh is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic t lymphocytes and nk cells, which results in proliferation of benign hemophagocytic histiocytes.
This means that to be affected, a person must have a change mutation in both copies of the responsible gene in each cell. Hlh occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. Hemophagocytic lymphohistiocytosis hlh is a disorder charecterised by immune dysregulation. There is uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes and their hematopoietic precursors in the bm giving rise to cytopenias. Hemophagocytic lymphohistiocytosis hlh is a rare but life threatening hyperinflammatory hypercytokinemia syndrome. Malignancyassociated hlh develops in patients with hematopoietic. 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. Between 1984 and 2006, we transplanted 18 consecutive patients with primary hemophagocytic syndromes. Hemophagocytic lymphohistiocytosis is an imperfect name for this syndrome or group of syndromes as hemophagocytosis is often absent and lymphohistiocytosis lacks precision. Hemophagocytic syndromes associated with other infectious illnesses, including sepsis, typhoid fever, tuberculosis, and leishmaniasis, may resolve with treatment of the underlying infection, and. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126. The genetic forms include familial disease and hemophagocytosis related to immune deficiencies, such as xlinked. Pdf hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the. Hayden a1, park s1, giustini d2, lee ay1, chen ly3. For claims with a date of service on or after october 1, 2015, use an equivalent icd10cm code or codes. Patients often suffer from recurrent fever, cytopenia, liver dysfunction, and a sepsislike syndrome that may rapidly progress to terminal multiple organ failure. Hemophagocytic lymphohistiocytosis is a lifethreatening disorder characterized by unbridled activation of. Hemophagocytic syndromes are found in all age groups. Hemophagocytic lymphohistiocytosis hlh, or haemophagocytic syndrome is a lifethreatening disease with a reported mortality of over 50% 3, belonging. Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of. Hlh may be diagnosed in association with malignant, genetic, or autoimmune. Familial hlh is caused by autosomalrecessive inheritance of gene mutations that cripple lymphocyte cytotoxicity. Phagocytosis of blood cells and their precursors is a hallmark of hemophagocytic syndromes.
Haemophagocytic syndromes haemophagocytic lymphohistiocytosis have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damagemainly reported in paediatric patients, but reports of adult presentation are increasing. Hemophagocytosis is achieved mostly by monocytes, macrophages, and nitroblue. For language access assistance, contact the ncats public information officer. Hemophagocytic syndromes hpss including hemophagocytic. Hereditary and acquired hemophagocytic lymphohistiocytosis. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a heterogenic syndrome, which leads to an acute. Arceci, in lanzkowskys manual of pediatric hematology and oncology sixth edition, 2016. Hemophagocytic lymphohistiocytosis hlh is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic t cells. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperinflammatory condition caused by a highly stimulated but ineffective immune response. The parents of an affected person usually each carry one mutated copy of the. The treatment and prognosis of patients with hlh and the macrophage activation syndrome mas, a form of hlh in patients with.
Hemophagocytic syndromes are characterized by impaired or absent function of natural killer nk cells and cytotoxic t cells and are generally divided into two categories. Hemophagocytic syndromes, also called hemophagocytic lymphohistiocytosis hlh have experienced a steadily growing attention over the past 10 years. Hemophagocytic lymphohistiocytosis with immunotherapy. Hemophagocytic lymphohistiocytosis hlh is a disorder characterized by systemic inflammation. If you have access to a journal via a society or association membership, please browse to your society journal, select an article to view, and follow the instructions in this box. Hemophagocytic syndrome an overview sciencedirect topics. This condition, which shares features with sepsis and systemic inflammatory response syndrome sirs, has received increasing attention in recent years such that 85% of the 1500 or so publications on hlh have appeared in the last decade. Hemophagocytic syndromes maggie davis hovda morning report 10302009 simple schematic showing that when nk and ctls can t effect their functions, the target. Prompt initiation of treatment for hlh is essential for the survival of affected patients. Hemophagocytic lymphohistiocytosis hlh, or haemophagocytic syndrome is a lifethreatening disease with a reported mortality of over 50% 3, belonging to the socalled cytokine storm syndromes. Infection and malignancyassociated hemophagocytic syndromes. Hemophagocytic lymphohistiocytosis definition, etiology, pathophysiology, primary and secondary hlh, clinical findings, investigations, diagnostic criteria, slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. More than 1500 publications on this topic have appeared since 2004.
It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated. Pdf hemophagocytic syndromes an update researchgate. When t cells and macrophages do not talk the hemophagocytic syndromes. Hemophagocytic lymphohistiocytosis hlh is an overwhelming clinical syndrome associated with extreme immune activation.
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