(MAS, acronym for Macrophage Activation Syndrome)

Non-neoplasic proliferation and excessive activation of the mononuclear phagocytosis system (monocytes and macrophages) which causes hemophagocytosis in the bone marrow and hematopoietic organs with important production of cytokines.

It is generally a process secondary to:

-         a viral  (EBV, CMV, herpes), bacterial, parasitic or fungal infection

-         a cancer, 

-         an autoimmune disease: systemic lupus erythematosus, juvenile polyarthritis

-         an immune deficiency: Griscelli syndrome or disease, Wiskott-Aldrich, Hermansky-Pudlak syndrome, Chediak-Higashi, X-linked lymphoproliferative syndrome

-         some medications (phenytoin). 

There is also a primary or family form linked to genes located on 9q and 10 p.

Fever, jaundice, splenomegaly, a maculo-nodular rash are the usual symptoms. There are sometimes meningeal signs and/or convulsions. May present as an atypical epiglottitis. 

Can start acutely with a fulminant evolution (hemophagocytic syndrome), often deadly, with DIC. A few cases of survival after ECMO have been reported.

Diagnostic criteria:

• familial form or known immune problem
• other forms: at least 1 of the following 5 criteria:

-         fever

-         splenomegaly

-         cytopenia affecting at least two cell lines

-         hypertriglyceridemia (inhibition of lipoprotein lipase) 

-         low fibrinogen (increase of plasminogen)

-         hemophagocytosis without sign of malignancy on biopsy (bone marrow, lymph node)

-         high ferritin (> 500 µg/l)

-         high level of soluble CD25 (part of interleukin-2Rα) (> 2400 U/ml)

Treatment: 

1)        infectious forms: treatment of infection ± dexamethasone;

2)         familial or genetic forms: bone marrow transplantation.

Anesthetic implications: 

check hemoglobin, platelets count and liver function. In case of hypertriglyceridemia, it is prudent to avoid the use of a continuous infusion of propofol.

References : 

-         Canbay O,  Celebi N, Karagoz AH, Saridemir B, Uzucugil F, Sahin A, Ayar U. 
Anesthetic management of a patient with hemophagocytic syndrome. 
Pediatr Anesth 2007 ; 812-4.

-         Rossetti E, Bianchi R, Chiusolo F, Onofri A, Picardo S.
Extracorporeal membrane oxygenation in a 10-year-old girl with macrophage activation syndrome. 
Pediatr Anesth 2012; 22: 937-8.

-         Gottschling S, Larsen R, Meyer S, Graf N, Reinhard H. 
Acute pancreatitis induced by short-term propofol administration. 
Pediatr Anesth 2005; 15: 1006-8.

Updated: January 2024