Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step

Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation

“…This is the first direct comparison demonstrating a significant survival advantage for patients undergoing RIC-HCT compared with MAC-HCT for definitive cure of HLH, which supports the initial observation of good outcomes of RIC-HCT for HLH reported by Cooper et al 16,17 We have observed that RIC eliminates the early mortality (Ͻ 180 days) that is often observed in patients with HLH undergoing MAC HCT, 21 and RIC significantly improves survival, with a 3-year survival rate of 92%. As in a previous cohort of HLH patients treated with RIC-HCT, 17 we observed no cases of hepatic veno-occlusive disease, which can occur in 5%-30% of patients with HLH undergoing MAC HCT, [8][9][10] and there were no cases of fatal pneumonitis/acute respiratory distress syndrome or fatal hyperinflammatory syndromes.…”

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

“…Therefore, the HLH-2004 criteria are used to diagnose MAS (see Table 2). [11] However, these criteria were developed primarily for pediatric patients and not specifically for MAS. Thus, it is not necessary for the criteria to be met in adults if the clinical presentation is highly suggestive of MAS.…”

Autoimmune-Associated Hemophagocytic Syndrome/Macrophage Activation Syndrome

“…The experience from the HLH-94 protocol (including etoposide and dexamethasone [DXM]) and other studies have led to the development of a new treatment protocol, HLH-2004 (including etoposide, DXM, CyA) (Henter et al, 2007). However, immunochemotherapy (i.e., HLH-94 andHLH-2004 protocols) is only temporarily effective in the control of FHL, and the outcome is uniformly fatal unless the patient undergoes allogeneic stem cell transplantation (alloSCT) (Jordan & Filipovich, 2008;Henter et al, 2007). Last but not least, since patients with HLH represent a unique population with high morbidity/mortality and disease-specific complications, consideration should be given to referring these patients to centers with significant experience in the treatment and care of HLH.…”