Macrophage Activation Syndrome

Sen, Ethan S; Clarke, Sarah L N; Ramanan, Athimalaipet V

doi:10.1007/s12098-015-1877-1

Cited by 49 publications

(64 citation statements)

References 38 publications

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“…Secondary, or acquired, HLH is triggered by a wide variety of infections, malignancies and autoimmune diseases. Secondary HLH in the context of rheumatic diseases, such as systemic juvenile idiopathic arthritis (sJIA), is often designated macrophage activation syndrome (MAS) 3 4. Although termed ‘secondary’, on some occasions HLH can be the presenting manifestation of the underlying condition in a previously well child.…”

Section: Introductionmentioning

confidence: 99%

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Diagnosing haemophagocytic syndrome

2016

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Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment. Distinguishing HLH from systemic sepsis can present a major challenge. Criteria for diagnosis and classification of HLH and MAS are available and a serum ferritin >10 000 µg/L is strongly supportive of HLH. Without early recognition and appropriate treatment, HLH is almost universally fatal. However, with prompt referral and advancements in treatment over the past two decades, outcomes have greatly improved.

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Section: Introductionmentioning

confidence: 99%

“…It will also provide a summary of epidemiology, aetiology and pathogenesis. For details on specific treatments of HLH and MAS, including immunosuppression, pro-apoptotic chemotherapy and haematopoietic stem cell transplantation, readers are referred to several recent reviews 1 2 4…”

Section: Introductionmentioning

confidence: 99%

Diagnosing haemophagocytic syndrome

2016

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“…Etoposide, a drug recommended in several treatment protocols for HLH, also carries with it several organ toxicities and is known to cause bone marrow suppression. [14] Anakinra is an IL-1 antagonist which has been studied extensively and found to be very effective in Adult Onset Still's Disease (AOSD), a process driven in part by IL-1b. [15] In AOSD complicated by MAS, anakinra resulted in complete resolution of MAS symptoms.…”

Section: Discussionmentioning

confidence: 99%

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Askin

Edelson

Dooley

et al. 2018

CRIM

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Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

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“…W związku z tym ważne jest, aby jak najszybciej postawić rozpoznanie/podejrzenie MAS, co nie zawsze jest proste i często prowadzi do opóźnionego włączenia prawidłowego leczenia [2,3,5,12,17,20,22,23]. TABELA 3.…”

Section: Opis Przypadkuunclassified

“…Dopiero w późniejszym okresie nabiera cech charakterystycznych dla obrazu szpiku w przebiegu zespołu hemofagocytarnego. Należy także pamiętać, że fagocytoza stwierdzona w badaniu szpiku nie jest kryterium obligatoryjnym dla postawienia rozpoznania [5,9,22].…”

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Zespół aktywacji makrofagów w chorobie Stilla u dorosłych – opis przypadku

Lewandowska

Brzosko

2017

Pomeranian J Life Sci

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Introduction: Macrophage activation syndrome (MAS) is a rare complication of autoimmune diseases. The background of the diseases is immune system dysfunction. The disease is characterized by varying symptomatology and the different dynamics of accumulation of symptoms. Case report: A female patient diagnosed with adult-onset Still's disease was admitted to the hospital because of fever, weakness, skin rash and dyspepsia which had lasted for several days. Diagnostic tests revealed the presence of anti-nuclear antibodies, pancytopenia, hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia, elevated activity of liver enzymes, and increased levels of C-reactive protein. The initial treatment with empirical antibiotic therapy and intravenous pulses of methylprednisolone was unsuccessful and provided no improvement. On the basis of bone marrow cytological examination, the diagnosis of MAS was established. The patient was treated with dexamethasone and cyclosporine A with improvement. The patient was followed up in the rheumatology out-patient department for 20 months without the relapse of MAS. Conclusion: Macrophage activation syndrome is a rare, dangerous complication of autoimmune diseases, requiring rapid diagnosis and aggressive treatment. Keywords: secondary haemophagocytic syndrome; haemophagocytic lymphohistiocytosis; adult-onset Still's diseases; dexamethasone; cyclosporine. ABSTRAKT Wstęp:Zespół aktywacji makrofagów (MAS) jest rzadkim powikłaniem chorób autoimmunologicznych. U jego podstawy leżą zaburzenia funkcjonowania układu immunologicznego. Symptomatologia może być różna, z różną dynamiką narastania objawów. Opis przypadku: Kobieta z ustalonym rozpoznaniem choroby Stilla u dorosłych została przyjęta do kliniki z powodu gorączki trwającej od 7 dni, osłabienia, wysypki obejmującej skórę całego ciała oraz objawów dyspeptycznych. W trakcie diagnostyki stwierdzono pancytopenię, hipertrójglicerydemię, hiperferrytynemię, hipofibrynogenemię, podwyższoną aktywność wszystkich enzymów wątrobowych, podwyższone stężenie białka C-reaktywnego, a także obecność przeciwciał przeciwjądrowych.W leczeniu początkowo zastosowano empiryczną antybiotykoterapię oraz pulsy metyloprednizolonu, co jednak nie przyniosło poprawy. Po otrzymaniu wyniku badania cytologicznego szpiku, w którym opisano pobudzoną fagocytozę mogącą odpowiadać zespołowi MAS, do leczenia włączono deksametazon oraz cyklosporynę A i wówczas stwierdzono poprawę. Chora zostawała pod stałą opieką przyklinicznej poradni reumatologicznej i od 20 miesięcy nie nastąpił nawrót MAS. Wnioski: Zespół aktywacji makrofagów jest rzadkim, groźnym powikłaniem chorób autoimmunologicznych wymagającym szybkiego rozpoznania i agresywnego leczenia.

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Macrophage Activation Syndrome

Cited by 49 publications

References 38 publications

Diagnosing haemophagocytic syndrome

Diagnosing haemophagocytic syndrome

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Zespół aktywacji makrofagów w chorobie Stilla u dorosłych – opis przypadku

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