What is hemophagocytic syndrome?

What is hemophagocytic syndrome?

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition caused by an overactive, abnormal response of the immune system. The immune system is the body's natural defense system against foreign or invading organisms or substances.

How is hemophagocytic syndrome treated?

Stem cell transplant can cure HLH in most cases. There is no way to prevent HLH, but as healthcare providers continue to learn more about it, treatment improves. Most children who are successfully treated go on to live normal lives.

Is hemophagocytic syndrome hereditary?

Hemophagocytic lymphohistiocytosis (HLH) may be inherited or acquired (due to non-genetic factors). Familial HLH is inherited in an autosomal recessive manner. This means that to be affected, a person must have a change ( mutation ) in both copies of the responsible gene in each cell .

What causes HLH disease?

Primary, or familial, HLH is caused by problems in genes that control how the immune system kills virus-infected or other abnormal cells in a person's body. These genes include PRF1, MUNC 13-4, STXBP2 and STX11. Primary HLH also occurs in some closely related inherited diseases.

How is hemophagocytic lymphohistiocytosis treated?

How We Treat Hemophagocytic Lymphohistiocytosis (HLH) Treatment should be started immediately once a diagnosis of HLH is established. Treatment includes dexamethasone and etoposide. Alternative “salvage” therapies that target T cells include anti-thymocyte globulin and alemtuzumab.

How long do people with HLH live?

Long-Term Outlook Familial HLH is fatal without treatment, with median survival of about two to six months.

Can HLH be caused by Covid?

HLH in COVID-19 positive patients is reported in various case reports [14,15]. However, the cases of sHLH in patients who have recovered from COVID-19 and are RAT and RT-PCR negative are sparse. Naous et al. reported a case of HLH as a cause of death in the post-COVID-19 patient [5].

Is HLH permanent?

HLH is a life-threatening condition. It can cause death in weeks or months if not treated. It may also cause death in months despite treatment.

Is HLH related to Covid?

HLH in COVID-19 positive patients is a recognized entity. However, in post-COVID-19 patients who have recovered and are negative by serological tests and reverse transcription-polymerase chain reaction test may present with sHLH due to dysregulation of the immune system.

How is hemophagocytic lymphohistiocytosis treated in children?

Treatment should be started immediately once a diagnosis of HLH is established. Treatment includes dexamethasone and etoposide. Alternative “salvage” therapies that target T cells include anti-thymocyte globulin and alemtuzumab.