EP18 Use of anakinra for haemophagocytic lymphohistiocytosis in adults
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DOI: 10.1093/rheumatology/keaa109.017
Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening syndrome characterised by severe immune activation. The primary form occurs in early childhood and is fatal without treatment. Secondary HLH can occur at any age, usually in… read more here.
Keywords: secondary hlh; treatment; haemophagocytic lymphohistiocytosis; none ... See more keywords
Etiologies and management of haemophagocytic lymphohistiocytosis: is it time for an updated protocol and targeted treatments?
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DOI: 10.1093/rheumatology/keaa741
Abstract: OBJECTIVE The objective of this study was to analyse the features, therapeutic approaches, and outcomes for adult patients with haemophagocytic lymphohistiocytosis (HLH) at a single centre. METHODS This study was a retrospective chart review of… read more here.
Keywords: 2004 protocol; total patients; haemophagocytic lymphohistiocytosis; hlh 2004 ... See more keywords
How i treat primary haemophagocytic lymphohistiocytosis
Sign Up to like & getrecommendations! Published in 2018 at "British Journal of Haematology"
DOI: 10.1111/bjh.15274
Abstract: Primary haemophagocytic lymphohistiocytosis (HLH) diseases are a collection of inherited genetic disorders that cause the syndrome of HLH. Great advances have been made in the last 20 years with regard to the discovery of many… read more here.
Keywords: primary haemophagocytic; haemophagocytic lymphohistiocytosis; treat primary; hlh diseases ... See more keywords
Haemophagocytic lymphohistiocytosis and Epstein-Barr virus: a complex relationship with diverse origins, expression and outcomes.
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DOI: 10.1111/bjh.17638
Abstract: Epstein-Barr virus (EBV) is a ubiquitous herpesvirus with rare but severe potential for lymphoproliferative complications. EBV is associated with a variety of presentations of haemophagocytic lymphohistiocytosis (HLH). HLH is a life-threatening hyperinflammatory syndrome that can… read more here.
Keywords: ebv; epstein barr; ebv associated; barr virus ... See more keywords
Retrospective single‐centre analysis of diagnostic approach to adult‐onset haemophagocytic lymphohistiocytosis
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DOI: 10.1111/imj.14891
Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a rare disorder with a poor prognosis characterised by substantial immune activation leading to end‐organ failure. In childhood, genetic defects that impair cytotoxic function of natural killer cells and T cells.… read more here.
Keywords: analysis diagnostic; lymphohistiocytosis; centre analysis; retrospective single ... See more keywords
Analysis of the clinical characteristics of lamotrigine‐induced haemophagocytic lymphohistiocytosis
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DOI: 10.1111/jcpt.13602
Abstract: Lamotrigine is currently known to be related to haemophagocytic lymphohistiocytosis (HLH). Knowledge regarding the association between HLH and lamotrigine is mainly based on case reports. The purpose of this study was to evaluate the clinical… read more here.
Keywords: analysis clinical; characteristics lamotrigine; lamotrigine induced; haemophagocytic lymphohistiocytosis ... See more keywords
HHV-8-associated haemophagocytic lymphohistiocytosis in a patient with advanced AIDS
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DOI: 10.1136/bcr-2017-222382
Abstract: We present a patient with advanced AIDS admitted with recurrent shock of unclear aetiology, fevers, altered mental status and refractory cytopenias. His case posed a diagnostic challenge because evaluation of septic shock in the setting… read more here.
Keywords: haemophagocytic lymphohistiocytosis; patient; advanced aids; hlh ... See more keywords
Natural killer/T-cell lymphoma and secondary haemophagocytic lymphohistiocytosis in pregnancy
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DOI: 10.1136/bcr-2018-224832
Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disorder. It is challenging to diagnose due to its rarity and variation in clinical presentation, laboratory abnormalities and underlying aetiologies. A reproductive-aged woman, gravida 2 para… read more here.
Keywords: haemophagocytic lymphohistiocytosis; lymphoma secondary; cell lymphoma;
Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles
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DOI: 10.1136/bcr-2018-227860
Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in… read more here.
Keywords: risk alleles; systemic lupus; lupus erythematosus; haemophagocytic lymphohistiocytosis ... See more keywords
Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery
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DOI: 10.1136/bcr-2019-230368
Abstract: Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism; however, MBD in a non-alcoholic diabetic patient has rarely been reported. The aetiology or pathophysiology of MBD is still unknown. A 50-year-old man with a… read more here.
Keywords: surgery; complication; disease; haemophagocytic lymphohistiocytosis ... See more keywords
Visceral leishmaniasis presenting as haemophagocytic lymphohistiocytosis
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DOI: 10.1136/bcr-2019-232576
Abstract: A 53-year-old man presented with a number of hospital admissions for investigation of fever of unknown origin. He became gradually weaker with significant weight loss, pancytopenia and progressive splenomegaly over a 6-month period of extensive… read more here.
Keywords: lymphohistiocytosis; leishmaniasis presenting; visceral leishmaniasis; investigation ... See more keywords