We present a consecutive series of 18 adults with HLH diagnosed at our institution between 2004 and 2009. All diagnoses were confirmed by pathology. The median age at diagnosis was 56 years (range: 18–73 years), with a male: female ratio of 2:1. Patients uniformly prese...
HLH in adults is often secondary to autoimmune diseases, cancer, or infections in contrast to familial HLH. Treatment of secondary HLH is directed against the triggering disease in addition to immunosuppressive therapy, the latter commonly according to the HLH-2004 protocol. Methods.We conducted a ...
The epidemiology of HLH varies considerably depending on the population heterogeneity and variable underlying triggers [6]. In fact, sHLH is one of the most fatal diseases in adults, with a mortality rate of up to 41%, but the prognosis also depends on the underlying triggers [6]. Owing ...
Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. In this case series, we present 7 cases of secondary HLH (sHLH) in adults with their presentation, course, and outcomes. We retrospectively looked at the 7 cases of secondary HLH who were diagnosed ...
However, we cannot eliminate the possibility that some patients will develop secondary HLH later on, e.g. as a result of bacterial sepsis or prolonged viral-CRS 44. Moreover, the judgment of the (un)favourable disease-courses based on clinical criteria reflects a rather weak clinical endpoint ...
There have been isolated reports of adults developing PIMS-TS, a rare inflammatory multisystem syndrome seen in children with COVID-19 which shares common features with Kawasaki disease, toxic shock syndrome and macrophage activation syndrome / sHLH. Here we present a case of COVID-19-associated ...
It is unclear if the improvement in disease markers was attributed to JAK inhibition alone. However, this experience combined with the positive in vivo murine data suggests that ruxolitinib may serve as a potential treatment option for HLH, pending the release of more robust data. To our ...
HLH can either be primary (resulting from inherited genetic mutations), or secondary (an inappropriate host response to infection, malignancy, or autoimmune disease or other triggers). Primary HLH tends to present in childhood or young adults, whereas secondary HLH presents in adults with a wide ...
Out of 451 standard-care SIRS patients, five patients had high Hscores (≥169), suggesting incipient or HLH-like disease, and these patients were in urgent need for intensified therapy. However, none of these patients fulfilled five HLH-2004 criteria required for formal diagnosis. From the ...
Risk factors may include male sex, presence of Crohn’s disease, and induction phase of treatment. Coagulation disorders are common during HLH and play a key role both in the global severity of the disease and in the occurrence of hemorrhagic complications [13]. Coagulation disorders confer a ...