Expert opinion on biological therapy · 2026
Current outlook on the use of biological agents to improve outcomes in adult secondary hemophagocytic lymphohistiocytosis.
СтатьяОбзорНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Ghonge Aishwarya, Samperio Victor M, Hamoud Moza, Dasanu Constantin A
Аннотация
INTRODUCTION: Adult secondary hemophagocytic lymphohistiocytosis (asHLH) is a fulminant hyperinflammatory condition triggered by cancers, infections, autoimmune conditions or drugs. The hallmark of its pathogenesis involves dysregulated immune effector cell activation and excessive cytokine release.
AREAS COVERED: This narrative review updates the reader on modern treatment options for asHLH. The traditional one-size-fits-all strategy derived from the pediatric HLH-2004 protocol is increasingly viewed as outdated today. A significant shift toward a more individualized, trigger- directed treatment approach has occurred in the HLH literature of recent years.
EXPERT OPINION: Apart from treating promptly the underlying trigger conditions, earlier incorporation of targeted anti-cytokine therapy such as anakinra is strongly favored these days. The cytotoxic etoposide shall be chiefly reserved for malignancy-associated HLH, including B-cell lymphomas, and refractory disease. Rituximab has led to superior outcomes when utilized to clear EBV-infected B-cell reservoirs. IVIG should be considered in infection-associated HLH. The anti-IFN-γ monoclonal antibody emapalumab has been approved for refractory HLH cases. The JAK 1/2 inhibitor ruxolitinib may prove useful in this space as well. The therapeutic arsenal is likely to evolve further in the direction of agents addressing specific pathophysiologic steps in asHLH. Targeting effector cytokines, their receptors, inflammasome pathways are promising future directions in asHLH therapeutics.
AREAS COVERED: This narrative review updates the reader on modern treatment options for asHLH. The traditional one-size-fits-all strategy derived from the pediatric HLH-2004 protocol is increasingly viewed as outdated today. A significant shift toward a more individualized, trigger- directed treatment approach has occurred in the HLH literature of recent years.
EXPERT OPINION: Apart from treating promptly the underlying trigger conditions, earlier incorporation of targeted anti-cytokine therapy such as anakinra is strongly favored these days. The cytotoxic etoposide shall be chiefly reserved for malignancy-associated HLH, including B-cell lymphomas, and refractory disease. Rituximab has led to superior outcomes when utilized to clear EBV-infected B-cell reservoirs. IVIG should be considered in infection-associated HLH. The anti-IFN-γ monoclonal antibody emapalumab has been approved for refractory HLH cases. The JAK 1/2 inhibitor ruxolitinib may prove useful in this space as well. The therapeutic arsenal is likely to evolve further in the direction of agents addressing specific pathophysiologic steps in asHLH. Targeting effector cytokines, their receptors, inflammasome pathways are promising future directions in asHLH therapeutics.