Hemophagocytosis is achieved mostly by monocytes, macrophages, and nitroblue. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a heterogenic syndrome, which leads to an acute. Hemophagocytic lymphohistiocytosis hlh, a rare but potentially fatal syndrome of immune hyperactivation, may be an underrecognized immunerelated adverse event irae. Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of. Infection and malignancyassociated hemophagocytic syndromes. Clinical characteristics and prognostic factors of adult. For language access assistance, contact the ncats public information officer. Hlh2004 diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Infectionand malignancyassociated hemophagocytic syndromes. This means that to be affected, a person must have a change mutation in both copies of the responsible gene in each cell. Hemophagocytic syndromes hemophagocytic lymphohistiocytosis, hlh represent a severe. Clinical features clinical criteria for the diagnosis of hlh, proposed by the histiocyte society 2, include clinical, laboratory, and histopathologic features table. Hemophagocytic lymphohistiocytosis hlh is a disorder characterized by systemic inflammation. Patients often suffer from recurrent fever, cytopenia, liver dysfunction, and a sepsislike syndrome that may rapidly progress to terminal multiple organ failure.
Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperinflammatory condition caused by a highly stimulated but ineffective immune response. When t cells and macrophages do not talk the hemophagocytic syndromes. Hemophagocytic lymphohistiocytosis hlh is a rapidly progressive, lifethreatening syndrome of excessive immune activation. Hemophagocytic syndromes associated with other infectious illnesses, including sepsis, typhoid fever, tuberculosis, and leishmaniasis, may resolve with treatment of the underlying infection, and. Familial hlh is caused by autosomalrecessive inheritance of gene mutations that cripple lymphocyte cytotoxicity. Hayden a1, park s1, giustini d2, lee ay1, chen ly3. Hemophagocytic lymphohistiocytosis with immunotherapy. Hemophagocytic lymphohistiocytosis is an imperfect name for this syndrome or group of syndromes as hemophagocytosis is often absent and lymphohistiocytosis lacks precision.
Characterized by benign proliferation of the mature histiocyte. Malignancyassociated hlh develops in patients with hematopoietic. Hemophagocytic lympohhistiocytosis and other hemophagocytic disorders. Biochemical markers include elevated ferritin and triglycerides, and low fibrinogen. 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. Hemophagocytic syndromes hpss including hemophagocytic. In 10 patients the diagnosis was fhlh, in 3 patients gs and in 5 patients chs. Hemophagocytic lymphohistiocytosis hlh, or haemophagocytic syndrome is a lifethreatening disease with a reported mortality of over 50% 3, belonging.
Hemophagocytic syndromes hpss including hemophagocytic lymphohistiocytosis hlh in adults. Hemophagocytic lymphohistiocytosis hlh is a hyperinflammatory syndrome with high mortality even with appropriate treatment. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a heterogenic syndrome, which leads to an acute, lifethreatening inflammatory reaction. Hemophagocytic syndrome an overview sciencedirect topics. Hemophagocytic lymphohistiocytosis hemophagocytic syndromes hemophagocytic lymphohistiocytosis hlh falls into two categories. Hemophagocytic lymphohistiocytosis hlh is an overwhelming clinical syndrome associated with extreme immune activation. If you have problems viewing pdf files, download the latest version of adobe reader. The genetic forms include familial disease and hemophagocytosis related to immune deficiencies, such as xlinked.
Hemophagocytic lymphohistiocytosis hlh is a lifethreatening hyperinflammatory syndrome and not an independent disease. Depending on the etiology, hlh can be divided into genetic i. Hemophagocytic syndromes and infection, beth israel deaconess medical center, boston, massachusetts, usa. Pdf hemophagocytic syndromes and infection researchgate. Pathophysiology and epidemiology of hemophagocytic. 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. Familial hlh is inherited in an autosomal recessive manner. Hlh occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. Between 1984 and 2006, we transplanted 18 consecutive patients with primary hemophagocytic syndromes. Patients with early relapse of primary hemophagocytic. Pdf hemophagocytic syndromes an update researchgate. Hemophagocytic syndromes hpss are rare, lifethreatening conditions characterized by.
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. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated. Pdf hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the. Hemophagocytic lymphohistiocytosis hlh may be inherited or acquired due to nongenetic factors. 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. There is uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes and their hematopoietic precursors in the bm giving rise to cytopenias. Hemophagocytic lymphohistiocytosis hlh has become more widely recognized in adults, with all ages affected. Hemophagocytic syndromes, also called hemophagocytic lymphohistiocytosis hlh have experienced a steadily growing attention over the past 10 years. In general, hlh is a syndrome of pathologic immune activation with clinical manifestations of extreme inflammation. Access to society journal content varies across our titles. Arceci, in lanzkowskys manual of pediatric hematology and oncology sixth edition, 2016. Hemophagocytic syndromes and infection volume 6, number.
Hemophagocytic lymphohistiocytosis hlh is a disorder charecterised by immune dysregulation. Hemophagocytic syndromes are found in all age groups. Recommendations for the management of hemophagocytic. More than 1500 publications on this topic have appeared since 2004. Hlh appears to affect all ages, although the hereditary and sporadic cases are reported primarily in children. Familial hemophagocytic lymphohistiocytosis genetic and. Discussion hemophagocytic syndromehemophagocytic lymphohistiocytosishlh it is a rare ds. Prompt initiation of treatment for hlh is essential for the survival of affected patients. Ppt hemophagocytic powerpoint presentation free to.
Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk. 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. Hemophagocytic syndrome hps, also known as hemophagocytic lymphohistiocytosis hlh, is a potentially lifethreatening immune system. Phagocytosis of blood cells and their precursors is a hallmark of hemophagocytic syndromes. Hemophagocytic lymphohistiocytosis hlh is a rare but life threatening hyperinflammatory hypercytokinemia syndrome.
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 lymphohistiocytosis hlh is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic t cells. Hemophagocytic lymphohistiocytosis linkedin slideshare. Hemophagocytic syndrome in children and adults springerlink. 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.
Ppt hemophagocytic syndromes powerpoint presentation. A free powerpoint ppt presentation displayed as a flash slide show on. Although ebv is the most common cause of infectionassociated hlh, recognition that at least some of the deaths associated with avian. 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. 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. For claims with a date of service on or after october 1, 2015, use an equivalent icd10cm code or codes.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126. Hereditary and acquired hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperin. Hemophagocytic syndromes in adult intensive care units. 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. The hlh disorders may be primary inheritedfamilial or secondary such as in response to specific types of infections infectionassociated hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis hlh is a lifethreatening syndrome that occurs as a complication in many clinical settings. 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.
Hlh may be diagnosed in association with malignant, genetic, or autoimmune. The parents of an affected person usually each carry one mutated copy of the. 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. Unlike other iraes, hlh triggered by immune checkpoint blockade is not well described. A set of diagnostic criteria was recommended by the histiocyte society for use in the hlh2004 research protocol, and this was revised in 2007. 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. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the. Hemophagocytic lymphohistiocytosis is a lifethreatening disorder characterized by unbridled activation of.
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