Sage-Tips

What is HLH test?

HLH can be diagnosed only with the proper tests. Blood tests include blood cell counts, liver function, infection studies, and markers of immune system activation such as ferritin and soluble IL-2 receptor levels. A bone marrow aspirate and biopsy may be performed to look for microscopic evidence of hemophagocytosis.

What is HLH in blood?

Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition in which certain white blood cells (histiocytes and lymphocytes) build up in and damage organs, including the bone marrow, liver, and spleen, and destroy other blood cells. HLH most commonly affects infants and young children.

Does HLH cause pain?

HLH patients have overactive histiocytes and lymphocytes, which are white blood cells that normally control infections, but when overactivated can cause swelling, redness, heat, pain and malfunction/damage of organs when they attack and accumulate in healthy lymph tissue (ed: Liver, Spleen, Lymph Nodes).

Is HLH chronic?

Conclusion. We have described a case of acquired HLH that presented in a fulminant form following a 3-year prodrome that was consistent with a mild, chronic form of HLH. Chronic HLH should be considered in the differential diagnosis of fever, splenomegaly and pancytopenia.

Are you born with HLH?

The genetic mutations that cause Primary HLH are present at birth, and patients often become ill with HLH in the first few years of life. If not detected and treated, primary HLH is usually fatal, typically within a few months.

Can the Covid vaccine trigger HLH?

HLH is a life-threatening hyperinflammatory syndrome caused by aberrantly activated macrophages and cytotoxic T cells, which may rapidly progress to terminal multiple organ failure. In this case, HLH was induced by the COVID-19 vaccination immuno-stimulation on a chronic EBV infection background.

What is the difference between “primary” and “ secondary” HLH?

Thus, the differentiation between “primary” and “secondary” HLH is falling out of vogue (because many patients who appear to have “secondary” HLH may actually have some underlying genetic mutations if you look carefully enough). ( 30766533) Some immunodeficiency syndromes or immunosuppressive agents may be associated with the development of HLH.

What is the difference between HLH Type 4 and Type 5?

Familial HLH, type 4 is caused by mutations in the STX11 gene. Familial HLH, type 5 is caused by mutations in the STXBP2 gene. All of the genes involved with HLH normally provide instructions for proteins that help destroy or turn off activated immune cells when they are no longer needed.

What is HLH (hemophagocytic lymphohistiocytosis)?

Hemophagocytic lymphohistiocytosis (HLH) is a condition in which the body makes too many activated immune cells (macrophages and lymphocytes). People with HLH usually develop symptoms within the first months or years of life.

Does hyperferritinemia always mean HLH?

More extreme elevations of ferritin are a bit more specific for HLH. However, there are many other causes of severely elevated ferritin (listed below). Therefore, extreme hyperferritinemia alone doesn’t necessarily indicate a diagnosis of HLH. Rapidly rising ferritin levels may be more consistent with evolving HLH.