We described a 9-year-old girl in whom MAS developed as a complication of systemic juvenile idiopathic arthritis (S-JIA) at onset with obvious hemophagocytosis presented in the marrow. [ncbi.nlm.nih.gov]

Case Presentation: A 33 year-old Hispanic male without significant history presented with an unremitting fever for 5 days, associated with myalgais, fatigue, night sweats, and a rash. [shmabstracts.com]

Enteric Fever Presenting as Hemophagocytic Lymphohistiocytosis (Macrophage Activation Syndrome). [webmedcentral.com]

• Increased Susceptibility to Infections

  Chronic granulomatous disease is a rare phagocyte disorder characterized by an increased susceptibility to infections and inflammatory complications. [ncbi.nlm.nih.gov]

• Tachypnea

  The boy was unconscious during examination (Glasgow coma score 4), hypotensive, had high grade fever, hemorrhagic skin rash, mucosal bleeding, tachycardia, tachypnea, hepatosplenomegaly and axillar lymphadenopathy. [signavitae.com]

• Onset in Adolescence

  Homepage Rare diseases Search Search for a rare disease Macrophage activation syndrome ORPHA:158061 Synonym(s): - Prevalence: Unknown Inheritance: - Age of onset: Childhood, Adolescent, Adult ICD-10: - OMIM: - UMLS: C1096155 MeSH: D055501 GARD: 12124 [orpha.net]

Macrophage activation syndrome workup. Medscape Reference 2012; 12. Available at: http://emedicine.medscape.com/article/1380671-workup [termedia.pl]

After extensive workup and lack of improvement with broad-spectrum antimicrobial therapy, intravenous immunoglobulin and steroids, the patient was diagnosed with macrophage activation syndrome after bone marrow biopsy and levels of soluble CD25 (soluble [ncbi.nlm.nih.gov]

[…] easy bruising and fatigue in addition high fever) Symptoms of disseminated intravascular coagulation (purpura, easy bruising, mucosal bleeding) CNS dysfunction, causing irritability, headache, disorientation, confusion, seizures, or coma Work-up The workup [xpertdox.com]

• Pericardial Effusion

  He developed pericardial effusion, hyponatremia and deranged renal function. Improvement on intravenous cortico steroids. High index of suspicion can lead to earlier diagnosis of macrophage activation syndrome. [ncbi.nlm.nih.gov]

Finally, we will review current treatment options for MAS and examine how cytokine-directed therapy could serve as novel treatment modalities. Copyright © 2014 Elsevier Ltd. All rights reserved. [ncbi.nlm.nih.gov]

CONCLUSIONS: Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis. Copyright © 2014 Elsevier España, S.L.U. All rights reserved. [ncbi.nlm.nih.gov]

The etiologies were: 5 AI, 5 HO, 2 Inf. and 1 Onc. disease. Hemophagocitc cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. [ncbi.nlm.nih.gov]

Etiology: MAS relates to abundant inflammation driven by a dysregulated immune response. [rheumaknowledgy.com]

The epidemiological and clinical features were collected during an epidemiological investigation. Public health measures were instituted by the Hellenic Centre for Disease Control and Prevention. [ncbi.nlm.nih.gov]

In adults, based on some limited epidemiologic studies, MAS is seen most frequently in association with adult onset Still’s disease, SLE, and various vasculitic syndromes. [the-rheumatologist.org]

This review summarizes the progress in understanding of MAS pathophysiology that may help define specific diagnostic biomarkers. [ncbi.nlm.nih.gov]

Pathophysiology In many cases a trigger is identified, often a viral infection, or a medication. [wikidoc.org]

Public health measures were instituted by the Hellenic Centre for Disease Control and Prevention. The Public Health intervention required the collaboration of epidemiologists, veterinarians and microbiologists. [ncbi.nlm.nih.gov]

Department of Preventive Medicine Cleveland Clinic Foundation Cleveland USA [doi.org]

This treatment significantly improved the condition of the child and in our opinion prevented a fatal outcome. keywords: macrophage, activation, syndrome references: Gietka P, Wieteska-Klimczak A, Smorczewska-Kiljan A, Rutkow-ska-Sak L. [termedia.pl]

CD163 found on macrophages is a haemoglobin scavenger receptor and has been widely described to be involved in the clearance of haemoglobin to prevent oxidative stress [ 51, 52 ]. [medsci.org]