Fever and Abdominal Pain

  • Esraa M. Eloseily
  • Randy Q. CronEmail author


  • Macrophage activation syndrome (MAS) or secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition

  • MAS is usually diagnosed in febrile intensive care unit patients

  • MAS can mimic septic shock, systemic inflammatory response syndrome (SIRS), or multi-organ dysfunction syndrome (MODS)

  • Timely diagnosis and early treatment are crucial to improve survival

  • Serum ferritin is usually highly elevated and will assist in recognizing secondary HLH/MAS in critically ill febrile patients

  • Bone marrow hemophagocytosis, although common, is not an essential feature for diagnosis, and its absence should not delay the start of an appropriate treatment

  • IL-1 inhibition with anakinra has been reported to be safe and efficacious in non-malignancy related forms of secondary HLH and MAS


Macrophage activation syndrome Hemophagocytic lymphohistiocytosis Systemic inflammatory response syndrome Multi-organ dysfunction syndrome 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of PediatricsUniversity of Alabama at BirminghamBirminghamUSA
  2. 2.Division of Rheumatology, Department of PediatricsAssiut University Children’s HospitalAssiutEgypt

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