This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis hlh, is a heterogenic syndrome, which leads to an acute, lifethreatening inflammatory reaction. Pathology outlines hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis hlh is a rare but lifethreatening hyperinflammatory. Virus associated hemophagocytic syndrome genetic and rare. Hemophagocytic syndrome natural killer cell cytotoxic t cell. Recent advances have been made in exploring other modalities of treatment. We are presenting two unusual cases of adults with haemophagocytic syndrome.
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 is an unusual syndrome characterized. Hlh can occur as a familial or sporadic disorder, and it can be triggered by a variety of. Hemophagocytic syndrome in a patient with acute human. The primary disease process causes increased production of stimulatory cytokines. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by. 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. Hereditary and acquired hemophagocytic lymphohistiocytosis ling zhang, md, jun zhou, md, and lubomir sokol, md, phd background. The hemophagocytic syndrome annals of internal medicine. This in turn raises the question of whether there are antibodies to the blood cells in the plasma, because macrophages have surface receptors for immunoglobulins. Hemophagocytic lymphohistiocytosis hlh is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis phagocytosis by macrophages of. Hemophagocytic lymphohistiocystosis johns hopkins medicine.
Subsequently, hlh has been reported in association with a variety of infections, and the term reactive hemophagocytic syndrome has been suggested to distinguish hlh associated with an identifiable infectious or noninfectious etiology from its hereditary forms. Dec 15, 2005 pediatric hemophagocytic syndrome hs is a severe and often fatal clinical disorder. Hemophagocytic lymphohistiocytosis hlh is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic t cells. 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. Pediatric hemophagocytic lymphohistiocytosis blood. This is a pdf file of an unedited manuscript that has been accepted for publication. Hemophagocytic syndrome an overview sciencedirect topics.
Hemophagocytic lymphohistiocytosis hlh is a severe. Hemophagocytic syndrome in children and adults springerlink. It is a lifethreatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated. Virus associated hemophagocytic syndrome genetic and. As a service to our customers we are providing this early version of the manuscript. Hemophagocytic syndromes and infection volume 6, number 6. Discussion of treatment is subdivided into fhl, infectionrelated, malignancyassociated, and. Hemophagocytic lymphohistiocytosis hlh is a rare disease that usually occurs in infants and young children. Unusual causes of haemophagocytic syndrome fatin al sayes, mrcpath, frcpc haemophagocytic syndrome is rare, often fatal clinical disorder in which release of inflammatory cytokines from macrophages results in marked haemophagocytosis. 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. Hemophagocytic syndrome in children and adults iwona malinowska, maciej machaczka, katarzyna popko, alicja siwicka, malgorzata salamonowicz, barbara nasilowskaadamska arch immunol ther exp warsz 2014. Hemophagocytic lymphohistiocytosis hlh is a condition in which the body makes too many activated immune cells macrophages and lymphocytes. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperinflammatory. Hemophagocytic syndromes histiocytosis association.
Hemophagocytic syndrome free download as powerpoint presentation. It most frequently affects infants from birth to 18 months of age, but the disease is also observed in children and adults of all ages. The mr imaging appearance of a case of virusassociated hemophagocytic syndrome complicated by diffuse cns infiltration is presented. Familial hemophagocytic lymphohistiocytosis genetic and. Hemophagocytic lymphohistiocytosis occurring as a primary or acquired disorder is a condition of chaotic and uncontrolled immune system stimulation.
The article on the acute lupus hemophagocytic syndrome 1 implies that the mechanism underlying the cytopenias is phagocytosis of the blood cells by macrophages. Unlike hemophagocytic histiocytic sarcoma, which is a neoplastic proliferation of phagocytic macrophages, hemophagocytic syndrome is secondary to an underlying disease, such as neoplasia, infection, or an immunemediated disorder. Recommendations for the management of hemophagocytic. Hemophagocytic lymphohistiocytosis hlh is a syndrome of severe immune activation with macrophage and tcell infiltration resulting in, multi organ damage. This retrospective analysis was performed at the university hospital charite universitatsmedizin berlin. This syndrome is frequently unrecognized, and thus, affected children may receive suboptimal management, leading to an increase in mortality. We gathered data from 556 out of 46,532 patients admitted to our anesthesiological icus between 2006 and 20, who had at least one plasma ferritin.
Hemophagocytic lymphohistiocytosis hlh is a syndrome describing patients with severe systemic hyperinflammation. If you have hlh, your bodys defense system, called your immune system, does not. Prompt treatment is critical, but the greatest barrier to a successful outcome is often a delay in diagnosis due to the rarity of this syndrome, variable clinical. Familial hemophagocytic lymphohistiocytosis tends to recur within the first year of life in 70 80% of cases am j clin pathol 20. Clinical manifestations include a fever, organ enlargement, and weight loss. Sep 11, 2017 try our interactive tool for help finding information, services, experts, financial aid, and more. Hemophagocytic lymphohistiocytosis hlh, also known as macrophage activation syndrome when occurring in the setting of a rheumatologic disorder, is a nonmalignant but oftenfatal disorder of immune dysregulation affecting multiple organs. Pdf hemophagocytic lymphohistiocytosis hlh is a disorder. It typically affects infants and young children but can be seen at all ages 5. How i treat hemophagocytic lymphohistiocytosis blood. The hyperinflammatory syndrome hemophagocytic lymphohistiocytosis can occur in the context of malignancies. Secondary hemophagocytic lymphohistiocytosis in adults clinical.
Hemophagocytic lymphohistiocytosis hlh is a rapidly progressive, lifethreatening syndrome of excessive immune activation. Introduction hemophagocytic syndrome is a rare disorder of the mononuclear phagocytic system. Hemophagocytic lymphohistiocytosis hlh is a severe hyperinflammatory syndrome induced by activated macrophages and cytotoxic t cells. Virusassociated hemophagocytic syndrome is a rare condition, precipitated by viral infection and characterized by proliferation of benign histiocytes with phagocytosis. Histiocyte society hlh2004 hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis with immunotherapy.
Hemophagocytic lymphohistiocytosis radiology reference. Hemophagocytic lymphohistiocytosis hlh is a rare disorder of the immune system primarily affecting young infants and children, although it can develop for the first time at any age. Pdf bacteriaassociated hemophagocytic syndrome juan. Approach to hemophagocytic syndromes histiocytosis association. The evaluation of patients presenting with a hemophagocytic syndrome should include serologic tests for systemic lupus erythematosus. Malignancytriggered hemophagocytic lymphohistiocytosis should be regarded seperately from hemophagocytic lymphohistiocytosis during chemotherapeutic treatment, which is frequently associated with an infectious trigger. Secondary hemophagocytic lymphohistiocytosis has a high mortality rate among adults despite recent advances in treatment. Clinical features and diagnosis of hemophagocytic lymphohistiocytosis. A multicenter retrospective analysis of 162 patients, the american journal of medicine 2014, doi. Hemophagocytic lymphohistiocytosis hlh is a potentially fatal hyperin. Hereditary and acquired hemophagocytic lymphohistiocytosis. Prompt initiation of treatment for hlh is essential for the survival of affected patients. Hemophagocytic syndrome associated with hepatitis a.
Hemophagocytic lymphohistiocytosis hlh is an aggressive and lifethreatening syndrome of excessive immune activation. Pediatric hemophagocytic syndrome hs is a severe and often fatal clinical disorder. Complete cases of common blood disorders peripheral blood, bone marrow, and diagnostic studies. Hlh occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. The treatment and prognosis of patients with hlh and the macrophage activation syndrome mas, a form of hlh in patients with. Clinical infectious diseases, jun 2004 castilletti concetta, preziosi roberta, bernardini giuliana, caterini antonio, gomes vito, calcaterra silvia, carletti fabrizio, capobianchi maria r. May 08, 20 virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Recognition of the acute lupus hemophagocytic syndrome and distinction from an infectionassociated hemophagocytic syndrome is important because it responds well to steroid therapy. The hlh2004 criteria remain as the common diagnostic guide. 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. A high index of suspicion is essential for early diagnosis and treatment. 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.
Unlike other iraes, hlh triggered by immune checkpoint blockade is not well described. Consensus recommendations for the diagnosis and management. Review the clinical features and epidemiology of hlh, its association with infection, and evidence that this syndrome results from disordered cellular immunity. Management of hemophagocytic lymphohistiocytosis hlh in. Lymphohistiocytosis hemophagocytic lymphohistiocytosis.
Hlh manifests recurrent fever, cytopenia, liver dysfunction, and a sepsislike syndrome that may complicated by multiple organ failure. Pdf hemophagocytic lymphohistiocytosis hlh is a lifethreatening hyperinflammatory syndrome and not an independent disease. Hemophagocytic syndrome in a patient with acute human immunodeficiency virus infection. Cns involvement of virusassociated hemophagocytic syndrome. Cytotoxic cells and macrophages cause multiorgan damage, hemophagocytosis, and severe systemic inflammation.
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 the body resulting in the destruction of. Hemophagocytic lymphohistiocytosis hlh is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. Hemophagocytic lymphohistiocytosis hlh, a rare but potentially fatal syndrome of immune hyperactivation, may be an underrecognized immunerelated adverse event irae. Apr 05, 2019 primary hemophagocytic lymphohistiocytosis ie, familial erythrophagocytic lymphohistiocytosis fel, an inherited form of hemophagocytic lymphohistiocytosis syndrome, is a heterogeneous autosomal recessive disorder found to be more prevalent with parental consanguinity.
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