Hemophagocytic syndrome remedies
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Clinic
- HLH is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterized by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines.
- HLH is classified as one of the cytokine storm syndromes.
- There are inherited and non-inherited (acquired) causes of HLH.
- Secondary HLH may also result from iatrogenic causes such as bone marrow or other organ transplantations; chemotherapy; or therapy with immunosuppressing agents
Signs and symptoms
- Onset of HLH occurs under the age of one year in approximately 70 %. Familial HLH should be suspected if siblings are diagnosed with HLH or if symptoms recur when therapy has been stopped.
- Familial HLH is an autosomal recessive disease, hence each sibling of a child with familial HLH has a 25% chance of developing the disease, a 50% chance of carrying the defective gene (which is very rarely associated with any risk of disease), and a 25% chance of not being affected and not carrying the gene defect.
- Patients with HLH, especially when untreated, may need intensive therapy. Therefore, HLH should be included in the differential diagnosis of intensive care unit patients with cytopenia and hyperferritinemia. Patients in the earlier stages of HLH are frequently hospitalized at internal medicine wards.