In the pediatric population, the incidence of HLH is thought to range from 1 to 225 per 300,000 live births, equally distributed between male and female infants, with the mean age of occurrence of 1.8 years. While the terms "primary" and "secondary" have been in use for some time, the North American Consortium for Histiocytosis recommended a new classification system that divides HLH into forms that respond to immunosuppressive treatment, which are referred to as "HLH disease" and forms that do not respond to immunosuppressives, which are referred to as "HLH mimics." Secondary, or acquired, HLH can be triggered by infection, malignancy, transplant, autoimmune disorders, or drugs. Primary HLH, also known as familial HLH (F-HLH), is caused by disease-causing variants in several genes. The Histiocyte Society established criteria for HLH for the HLH-2004 clinical trial, and these criteria are often referred to by physicians considering a diagnosis of HLH. Patients often have elevated ferritin and soluble interleukin-2 receptor concentrations, as well as low fibrinogen levels. Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder characterized by fever, cytopenias, coagulopathy, hepatosplenomegaly, neurologic symptoms, and hemophagocytosis in the bone marrow, spleen, lymph nodes, or liver. Specimen Stability Information : Ambient (preferred)/Refrigerated (<24 hours) Cultured cells from a prenatal specimen will not be accepted. An additional 3 to 4 weeks is required to culture fibroblasts before genetic testing can occur.Ĭollection Instructions: Submit confluent cultured fibroblast cells from a skin biopsy from another laboratory. Specimen Stability Information : Refrigerated (preferred)/AmbientĪdditional Information: A separate culture charge will be assessed under CULFB / Fibroblast Culture for Biochemical or Molecular Testing. The solution should be supplemented with 1% penicillin and streptomycin. Supplies : Fibroblast Biopsy Transport Media (T115)Ĭontainer / Tube: Sterile container with any standard cell culture media (eg, minimal essential media, RPMI 1640). Specimen Stability Information: Ambient (preferred) 4 days/Refrigerated Send whole blood specimen in original tube. Preferred: Lavender top (EDTA) or yellow top (ACD)Ģ. Submit only 1 of the following specimens: Call 80 for instructions for testing patients who have received a bone marrow transplant. Patient Preparation: A previous bone marrow transplant from an allogenic donor will interfere with testing.
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