The disease is potentially serious. In the event of a legitimate suspicion, admission to a pediatric or medical department in hospitals with the option of intensive care is recommended.
Examples of disease signs that require treatment (from UpToDate 2013):
- Patient with CNS symptoms, cytopenias, coagulopathy and ferritin above 3000 ng / mL or rapidly increasing ferritin or increased sCD25 (soluble IL-2 receptor)
- Patient with hypotension, fever, without response to broad spectrum antibiotics and ferritin over 3000 ng / mL or rapidly increasing ferritin or increased sCD25
- Methylprednisolone IV in 3 consecutive days
- High dose corticosteroids are adequate treatment in excess of 50%
- If CNS affection is dexamethasone relevant (the drug crosses the blood-brain barrier)
- Fresh frozen plasma if low fibrinogen and threatening bleeding
- Sepons all antirevulsive medication other than corticosteroids / prednisolone 4mg / kg / d / dexamethasone 6mg / m2 / day
- Cyclospirine-A 2 - 5mg / kg / day can be used
- Intravenous immunoglobulin (IVIG) can be considered
- Etoposide (Etopophos) is a cytostatics used abroad. Not registered in Norway
- Cyclofosfamine (Sendoxan) is an alternative
- Transition to methotrexate for maintenance therapy if needed
- Transfusion if hemoglobin falls (and not contraindications) or if platelets lower than 50.000
Interlevkin-1 inhibitors (Kineret) also have an effect in some cases.
For children with MAS / HLH, one can follow HLH-2004 protocol based on a study that has been ongoing since 2004-2017.
- Take daily blood tests initially
- Follow clinical signs (temperature, rash, lymphadenopathy, hepatosplenomegaly, neurological symptoms)
- Bloodcell counts
- Coagulation: INR, fibrinogen, D-dimer
- Ferritin, renal function, electrolytes
- Liver enzymes
Recommended Literature: Rosee PL, 2019 (adults)