2017
DOI: 10.1002/cncr.30826
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A consensus review on malignancy‐associated hemophagocytic lymphohistiocytosis in adults

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. In this review we focus on malignancy-associated … Show more

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Cited by 181 publications
(241 citation statements)
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“…50 To determine whether a patient has an inherited form of HLH, genetic analyses (ie, next-generation sequencing, gene panels, whole-exome sequencing), along with functional testing (ie, NK cell cytotoxicity and CD107a upregulation), will be needed. 10,[15][16][17]46,51 This is particularly true for patients with a family history of consanguinity and/or HLH, partial albinism, or recurrent disease and for young male adults with Epstein-Barr virus (EBV) infection or lymphoproliferation. Testing should also be considered for HLH patients with unknown trigger.…”
Section: Statementmentioning
confidence: 99%
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“…50 To determine whether a patient has an inherited form of HLH, genetic analyses (ie, next-generation sequencing, gene panels, whole-exome sequencing), along with functional testing (ie, NK cell cytotoxicity and CD107a upregulation), will be needed. 10,[15][16][17]46,51 This is particularly true for patients with a family history of consanguinity and/or HLH, partial albinism, or recurrent disease and for young male adults with Epstein-Barr virus (EBV) infection or lymphoproliferation. Testing should also be considered for HLH patients with unknown trigger.…”
Section: Statementmentioning
confidence: 99%
“…In highly active HLH, or if severe organ damage is imminent, dose-adjusted etoposide (50-100 mg/m 2 ) may be used prior to tumor-specific treatment. 16 Etoposide can be added to CHOP or CHOP-like protocols (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisone [CHOEP] or doseadapted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin [DA-EPOCH]). 9 Patients with aggressive lymphomas should be considered for evaluation of involvement of the central nervous system (CNS), with cerebral magnetic resonance imaging and lumbar puncture.…”
Section: Malignancy-triggered Hlhmentioning
confidence: 99%
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“…In adults, all of the studies examining treatment of secondary HLH have included fewer than 20 patients and have been retrospective in nature 15. Therefore, expert guidelines generally recommend adaptation of the HLH-94 regimen to treat adult patients 19. Our patient’s clinical presentation was felt to be most consistent with secondary HLH, and her treatment plan was therefore designed to approximate the HLH-94 regimen rather than therapies recommended for treatment of CRS.…”
Section: Discussionmentioning
confidence: 99%
“…Antithymocyte globulin has also been used in combination with steroids, cyclosporine and intrathecal chemotherapy [11]. A recent consensus review on malignancy-associated HLH suggests tailoring treatment to the underlying trigger, performance status, organ function and additional therapies the patient is receiving [12]. Early consultation with a hematologist and center experienced in the treatment of HLH is critical, both because immunomodulatory therapy for HLH is an area of active investigation, and because bone marrow transplantation is considered the curative therapy for patient with a known or suspected genetic cause of HLH.…”
Section: Hlh Treatment Treatmentmentioning
confidence: 99%