Search
macrophage activation syndrome
Etiology:
- complication of rheumatoid disease
- systemic juvenile idiopathic arthritis
- adult-onset Still disease
- systemic lupus erythematosus
- Kawasaki disease
- viral infections (influenza, Herpes simplex, & EBV)
Pathology:
- hepatosplenomegaly
- hepatic insufficiency
- coagulopathy
- neurologic disorder
- activation & uncontrolled proliferation of T-cells & well-differentiated macrophages, leading to widespread hemophagocytosis & cytokine overproduction
- decreased NK-cell cytolytic function resulting from decreased perforin gene (PRF1) expression
- expansion of cytotoxic T lymphocytes that produce macrophage- activating cytokines, including IFN-gamma
- alternative pathway of macrophage differentiation
- elevated IL-18 in serum
Laboratory:
- complete blood count: pancytopenia
- liver function tests
- serum transaminases may be moderate elevated
- serum ferritin: very elevated [4]
- prothrombin time, INR often prolonged
- aPTT often prolonged
- D-dimer: fibrin degradation products often present
- plasma fibrinogen: hypofibrinogenemia
- serum triglycerides: hypertriglyceridemia
- IL-18 in serum elevated
Complications:
- may be rapidly fatal (8-60%) [4]
Management:
- parenteral administration of high doses of corticosteroids
- high-dose intravenous immunoglobulins, cyclophosphamide, plasma exchange, & etoposide has been used with conflicting results
- cyclosporine A may be useful in corticosteroid-resistant disease
- stem cell transplantation provides only long-term disease-free survival
- consults:
- rheumatology
- hematology/oncology
- infectious disease
Specific
hemophagocytic lymphohistiocytosis; hemophagocytic syndrome
General
leukocyte disorder
syndrome
References
- Ravelli A et al
eMedicine: Macrophage Activation Syndrome
http://emedicine.medscape.com/article/1380671-overview
- Ravelli A
Macrophage activation syndrome.
Curr Opin Rheumatol. 2002 Sep;14(5):548-52.
PMID: 12192253
- Sawhney S et al
Macrophage activation syndrome: a potentially fatal
complication of rheumatic disorders
Arch Dis Child 2001;85:421-426
PMID: 11668110
http://adc.bmj.com/content/85/5/421.abstract
- Cho J, Jong SC, Ng SB et al
A Woman With Fever and Lymphadenopathy.
JAMA. 2018;319(24):2552-2553.
PMID: 29946706
https://jamanetwork.com/journals/jama/fullarticle/2685964