acquired hemophagocytic syndrome in a patient with synovial sarcoma: a case report

What is hemophagocytic syndrome?

Hemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome that can be fatal. This syndrome can sometimes occur in normal people with medical problems that can cause a strong activation of the immune system, such as infection or cancer.

What causes hemophagocytic syndrome?

General Discussion. 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 diagnosed?

How is HLH diagnosed? Your healthcare provider bases a diagnosis of HLH on your symptoms, physical exam findings, and several lab tests. A prolonged fever is a commonly occurring symptom. An enlarged liver or spleen (located in the upper left side of the abdomen) are key physical findings.

How is hemophagocytic syndrome treated?

Treatment includes targeting the underlying disorder and controlling the immune dysregulation. Etoposide, dexamethasone, and ultimately HSCT are the mainstay for the majority of patients with HLH. Further studies are required to improve our understanding of the optimal treatment of HLH in adults.

When do you suspect HLH?

When to suspect HLH? Clinically, acquired and genetic forms of HLH are often indistinguishable and one should have a high index of suspicion for any critically ill patient who presents with prolonged fever and unexplained cytopenia's with multi-organ involvement.

How many cases of HLH are there?

How common is HLH? HLH is diagnosed in fewer than 1 out of every 50,000–100,000 children per year. The condition occurs equally often in boys and girls. Since HLH causes symptoms similar to many other disorders, doctors think that more children develop HLH than are recognized and reported.

What is the survival rate of HLH?

Familial HLH is fatal without treatment, with median survival of about two to six months. Chemotherapy and/or immunotherapy temporarily control the disease, but symptoms inevitably return.

What is the difference between HLH and MAS?

MAS is a disorder related to hemophagocytic lymphohistiocytosis (HLH), which is divided into primary and secondary HLH. Primary or familial HLH is an inherited disease, whereas secondary HLH is triggered by other diseases, including infections, malignancy, and autoimmune diseases.

How does HLH affect the brain?

With HLH, histiocytes become overactive and begin to attack the body's own tissues as if they are invading organisms. The result can be damage to organs such as the brain, liver and bone marrow. HLH causes histiocytes to release chemicals into the blood and ingest other cells, mistaking them for an infection.

How long can adults live with HLH?

Adult HLH is a rare and almost universally fatal disease entity without treatment with published median survival of 1.8–2.2 months.

Is HLH an autoimmune disease?

According to common classifications, HLH does not fulfill the criteria of an autoimmune disease (ie, an immune reaction to a more or less defined self-antigen).

Is HLH reversible?

We demonstrated that PRES is reversible in patients with HLH and that these patients have favorable outcomes with prompt diagnosis and treatment.

How long do you live with HLH?

Familial HLH is fatal without treatment, with median survival of about two to six months. Chemotherapy and/or immunotherapy temporarily control the disease, but symptoms inevitably return. Stem cell transplant is the only way to cure familial HLH.

What causes death in HLH?

Therefore, the cause of death was deemed to be HLH triggered by SFTSV infection. HLH is a life-threatening disease that is caused by excessive activation of the immune system and is associated with many underlying conditions (8,9).

How long can adults live with HLH?

Adult HLH is a rare and almost universally fatal disease entity without treatment with published median survival of 1.8–2.2 months.

Does Covid cause HLH?

COVID-19-associated haemophagocytic lymphohistiocytosis (HLH) can present as a postacute COVID-19 syndrome, even after a pauci-symptomatic initial infection. The development of HLH after a SARS-CoV-2 infection is rare and represents an aberrant virus–host interaction.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen.

Case presentation

A 20-year-old man, affected by synovial sarcoma of the left buttock with pulmonary metastases since October 2013, was treated with doxorubicin and ifosfamide as first-line chemotherapy.

Conclusion

In conclusion, we report a noteworthy case of HLH secondary to chemotherapy for synovial sarcoma that achieved prompt remission with steroids, highlighting the importance to recognize a, albeit rare, correlation between HLH and solid tumors.

Similar Articles

To arrive at the top five similar articles we use a word-weighted algorithm to compare words from the Title and Abstract of each citation.