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Macrophage Activation Syndrome

MAS


Presentation

  • 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]
Splenectomy
  • After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue.[ncbi.nlm.nih.gov]
  • […] of refractory disease despite eventual progression to cyclosporine A/etoposide, the suggested protocol of Emmenegger et al. [ 12 ] allows for consideration of progression to such therapies as polychemotherapy, plasma exchange, antithymocyte globulin, splenectomy[karger.com]
Turkish
  • An 11-year-old Turkish girl from a non-consanguineous family was suffering from joint pain, fever, hepatosplenomegaly, and respiratory insufficiency.[ncbi.nlm.nih.gov]
Noncompliance
  • Subsequent recurrences may have occurred because of the family's noncompliance with treatment.[ncbi.nlm.nih.gov]
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]
Veterinarian
  • The Public Health intervention required the collaboration of epidemiologists, veterinarians and microbiologists. Emphasis was given to communication activities and misconceptions concerning canines and their role in the disease.[ncbi.nlm.nih.gov]
Respiratory Insufficiency
  • An 11-year-old Turkish girl from a non-consanguineous family was suffering from joint pain, fever, hepatosplenomegaly, and respiratory insufficiency.[ncbi.nlm.nih.gov]
Odynophagia
  • Ten days later, she had a spiking fever, dyspnea, low back pain, abdominal pain, odynophagia, and hepatomegaly.[ncbi.nlm.nih.gov]
Cranial Nerve Involvement
  • Rumbach L, Berger E, Tatu L et al (1997) Transient multiple cranial nerve involvement as a first sign of macrophage activation syndrome. J Neurol Neurosurg Psychiatry 62(3):292–293 CrossRef PubMed Google Scholar 4.[doi.org]
Incontinence
  • CASE REPORT A 38-year-old male patient presented with severe muscle weakness, diffuse abdominal cramps with vomiting and incontinence of stool, confusion, cough, and sweating increasing in severity for about 1 week.[ncbi.nlm.nih.gov]
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]

Workup

  • 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]
  • Macrophage activation syndrome workup. Medscape Reference 2012; 12. Available at:[termedia.pl]
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]
Atelectasis
  • Chest X-ray showed bilateral pleural effusions, atelectasis and infiltrates suggestive of volume overload. The patient continued to have significant leukocytosis, anemia and thrombocytopenia. Ferritin levels also remained elevated (1349 μg/L).[academic.oup.com]
Reticulocyte Count Decreased
  • Shortly after engraftment with UCB, these mice develop a severe, fatal macrophage activation syndrome (MAS) characterized by a progressive drop in rbc numbers, increased reticulocyte counts, decreased rbc half-life, progressive cytopenias, and evidence[ncbi.nlm.nih.gov]

Treatment

  • 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]

Prognosis

  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

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

Prevention

  • 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]
  • Hospital for Sick Children Health A-Z Search a complete list of child health articles expand_more View All Drug A-Z Search a list of articles about medications expand_more View All Learning Hubs Browse a complete list of content groups Healthy Living & Prevention[aboutkidshealth.ca]
  • 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]

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